Publications by authors named "Luc Mouthon"

417 Publications

Efficacy of baricitinib for refractory large vessel vasculitis.

Rheumatology (Oxford) 2021 Jul 10. Epub 2021 Jul 10.

Service de médecine interne, Centre de référence maladies auto-immunes et systémiques rares d'ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.

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http://dx.doi.org/10.1093/rheumatology/keab541DOI Listing
July 2021

Efficacy and safety of treatments in cutaneous polyarteritis nodosa: a French observational retrospective study.

J Am Acad Dermatol 2021 Jul 2. Epub 2021 Jul 2.

Service de Médecine Interne, Centre de Référence Maladies Autoimmunes et Systémiques Rares, Hôpital Cochin, APHP-Centre Université de Paris, F-75014 Paris; Université de Paris, F-75006, Paris. Electronic address:

Background: Cutaneous polyarteritis nodosa (cPAN) is a form of medium-sized vessel vasculitis. Despite a disabling and prolonged course, data on treatment efficacy and safety remain scarce.

Objectives: We aimed to describe treatment efficacy and safety in patients with cPAN.

Methods: This was a multicentre retrospective observational study, recording clinical and biological data together with treatments received. The primary outcome was the rate of complete response (CR) at month 3. Secondary outcomes included drug survival, and safety was assessed.

Results: We included 68 patients who received a median of 2 therapeutic lines (interquartile range 1-3). Overall, 13/42 (31%) patients achieved CR with colchicine, 4/17 (23%) with dapsone, 11/25 (44%) with glucocorticoids (GCs) alone, 1/9 (11%) with NSAIDs, 11/13 (84%) with GCs+azathioprine (AZA) and 7/15 (47%) with GCs+methotrexate. GCs+AZA had the best drug survival (median duration 29.5 months [IQR 19.5-36.0]). Response at month 3 was decreased with peripheral neurological involvement (odds ratio 0.19 [95% confidence interval 0.03-0.81], p=0.04). Overall, the rate of treatment-related adverse events was 18%, which led to treatment discontinuation in 7% of patients.

Limitation: Retrospective study CONCLUSION: Colchicine seems to confer good benefit-risk balance in cPAN without peripheral sensory neuropathy. GCs+AZA seems the best treatment for disease relapse.
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http://dx.doi.org/10.1016/j.jaad.2021.06.872DOI Listing
July 2021

Quantification of Antifibrillarin (anti-U3 RNP) Antibodies: A New Insight for Patients with Systemic Sclerosis.

Diagnostics (Basel) 2021 Jun 9;11(6). Epub 2021 Jun 9.

Internal Medicine Department, North Hospital of Marseilles, Public Assistance Hospital of Marseilles (AP-HM), 13015 Marseilles, France.

Background: The detection of additional autoantibodies is of great concern in systemic sclerosis (SSc) when those included in the ACR/EULAR classification are negative. In this context, the interest of antifibrillarin (anti-U3RNP) autoantibodies (AFAs) in the routine evaluation of SSc remains unclear. We aimed to assess the relevance of AFAs and their clinical association in SSc patients.

Methods: In a multicenter observational retrospective study, we collected immunological and clinical data associated with AFA positivity in SSc ( = 42) and non-SSc patients ( = 13). Patients with SSc negative for AFAs ( = 83) were considered as a control group. AFAs were detected by indirect immunofluorescence (IIF) using HEp-2 cells, EliA or immunoblot techniques.

Results: We confirmed a typical nuclear IIF pattern and showed that AFAs are mostly exclusive towards SSc conventional autoantibodies. Although also observed in non-SSc patients, high levels of AFAs with the ELiA technique allowed the diagnosis of SSc. Compared to AFA-negative SSc patients, AFA-positive SSc patients more frequently exhibited visceral involvements. They more frequently suffered from the diffuse cutaneous form and had a higher global severity of the disease.

Conclusions: We demonstrate the usefulness of quantifying AFAs in the immunological exploration of SSc, especially when patients are seronegative for SSc conventional autoantibodies and display a typical IIF pattern. AFAs might constitute an interesting marker of SSc severity.
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http://dx.doi.org/10.3390/diagnostics11061064DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8226926PMC
June 2021

Revisiting characteristics, treatment and outcome of cardiomyopathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

Rheumatology (Oxford) 2021 Jun 22. Epub 2021 Jun 22.

Université Paris Descartes, Sorbonne Paris Cité, Paris, France.

Objectives: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing eosinophil-rich vasculitis. Specific cardiomyopathy (CM) was described in old studies as the most important predictor of mortality. We aimed to revisit EGPA-related CM and its outcome in recent decades.

Methods: We reviewed all EGPA patients managed from 2000 to 2019 in our vasculitis clinic. Baseline characteristics and outcomes were analyzed. EGPA-related CM was defined as clinical or extra-clinical manifestations of patent myocardial involvement, after exclusion of other causes.

Results: We included 176 patients. Median age was 47 years (IQR 36-58 years). Specific CM was observed in 70 patients (40%). Cardiac symptoms were observed in 81% of CM+ patients, including mainly typical or atypical chest pain and peripheral edema. Abnormal EKG, TTE and cardiac magnetic resonance imaging (CMRI) were found in 72%, 72% and 99% in CM+ patients, respectively, contrasting with abnormalities in 32%, 38% and 60% in CM-negative patients. Late gadolinium enhancement (LGE) was the most frequent abnormality on CMRI (70%). CM+ patients were less frequently ANCA-positive, had less frequent peripheral neuropathy and had higher eosinophil count. Major adverse cardiovascular events (MACE) occurred in 13%, both in CM+ and CM- patients. Abnormal EKG and LGE on CMRI were associated with the occurrence of MACE. Four patients died, but none from cardiac causes.

Conclusions: Specific cardiomyopathy is frequent in EGPA, especially in ANCA-negative patients with high eosinophil counts. Long-term outcome was better than previously reported. Abnormal EKG and LGE on CMRI were associated with the occurrence of MACE.
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http://dx.doi.org/10.1093/rheumatology/keab514DOI Listing
June 2021

Nonsystemic vasculitic neuropathy: Presentation and long-term outcome from a French cohort of 50 patients.

Autoimmun Rev 2021 Aug 9;20(8):102874. Epub 2021 Jun 9.

Department of Neurology, Hôpital Henri Mondor, Créteil, France; Department of Internal Medicine, National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, Paris. Electronic address:

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http://dx.doi.org/10.1016/j.autrev.2021.102874DOI Listing
August 2021

Drug-induced eosinophilic fasciitis: A dual pharmacovigilance analysis.

J Am Acad Dermatol 2021 May 27. Epub 2021 May 27.

Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université de Paris, Paris, France.

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http://dx.doi.org/10.1016/j.jaad.2021.05.030DOI Listing
May 2021

Ocular Safety Profile of BRAF and MEK Inhibitors: Data from the World Health Organization Pharmacovigilance Database.

Ophthalmology 2021 May 14. Epub 2021 May 14.

Service de Médecine Interne, Centre de Référence des maladies auto-immunes systémiques rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France; Université de Paris, F-75006, Paris, France. Electronic address:

Purpose: BRAF inhibitors (BRAFi) and MEK inhibitors (MEKi) significantly improved metastatic melanoma prognosis. Ocular adverse effects (OAEs) represent an uncommon but disabling toxicity of these drugs. We aimed to characterize the ocular safety profile of BRAFi or MEKi and to detect possible safety signals.

Methods: We performed a retrospective, observational, pharmacovigilance study using VigiBase, the World Health Organization global safety database. Ocular adverse effects were classified according to the eye segments and the inflammatory pattern based on the Standardization of Uveitis Nomenclature. Associations among BRAFi monotherapy, MEKi monotherapy, and BRAFi+MEKi combination therapy and OAE reporting were assessed using disproportionality analysis. Results were expressed with the reporting odds ratio (ROR) and its 95% confidence interval (CI).

Results: From January 2010 to October 2019, 1568 OAE cases were reported with BRAFi or MEKi. Among them, 1006 cases with sufficient data were included, corresponding to 310 (30.8%), 124 (12.3%), and 572 (56.9%) cases reported with BRAFi, MEKi, or BRAFi+MEKi combination therapy, respectively. BRAF inhibitor monotherapy was significantly associated with the reporting of iris and ciliary body abnormalities (ROR, 8.7; 95% CI, 6.0-12.5), diffuse abnormalities (ROR, 7.1; 95% CI, 5.4-9.4), anterior uveitis (ROR, 8.6; 95% CI, 6.0-12.1), and panuveitis (ROR, 7.1; 95% CI, 5.4-9.4). MEK inhibitor monotherapy was associated with the reporting of retinal and choroid abnormalities (ROR, 9.5; 95% CI, 7.4-12.2), diffuse abnormalities (ROR, 2.5; 95% CI, 1.1-6.1), and panuveitis (ROR, 2.5; 95% CI, 1.1-6.1). Combinations of BRAFi and MEKi therapies were associated with OAEs from both drugs, with a possible synergistic or additive effect for diffuse abnormalities and panuveitis.

Conclusions: Our study characterizes the ocular safety profile of BRAFi and MEKi. We identify possible safety signals for several OAEs not previously reported with BRAFi and MEKi. Our data provide the rationale for a personalized management of OAE in patients with BRAFi+MEKi combination therapy according to the type of ocular reaction.
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http://dx.doi.org/10.1016/j.ophtha.2021.05.008DOI Listing
May 2021

A role for IL-33-activated ILC2s in eosinophilic vasculitis.

JCI Insight 2021 Jun 22;6(12). Epub 2021 Jun 22.

Department of Internal Medicine, National Referral Center for Rare and Systemic Autoimmune Diseases, Cochin Hospital, AP-HP, Paris, France.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare but serious disease with poorly understood mechanisms. Here, we report that patients with EGPA have elevated levels of TSLP, IL-25, and soluble ST2, which are well-characterized cytokine "alarmins" that activate or modulate type 2 innate lymphoid cells (ILC2s). Patients with active EGPA have a concurrent reduction in circulating ILC2s, suggesting a role for ILC2s in the pathogenesis of this disease. To explore the mechanism of these findings in patients, we established a model of EGPA in which active vasculitis and pulmonary hemorrhage were induced by IL-33 administration in predisposed, hypereosinophilic mice. In this model, induction of pulmonary hemorrhage and vasculitis was dependent on ILC2s and signaling through IL4Rα. In the absence of IL4Rα or STAT6, IL-33-treated mice had less vascular leak and pulmonary edema, less endothelial activation, and reduced eotaxin production, cumulatively leading to a reduction of pathologic eosinophil migration into the lung parenchyma. These results offer a mouse model for use in future mechanistic studies of EGPA, and they suggest that IL-33, ILC2s, and IL4Rα signaling may be potential targets for further study and therapeutic targeting in patients with EGPA.
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http://dx.doi.org/10.1172/jci.insight.143366DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8262498PMC
June 2021

The Clinical Spectrum and Outcome of Uveomeningitis: A Comprehensive Analysis of 110 Cases.

Ocul Immunol Inflamm 2021 May 11:1-6. Epub 2021 May 11.

Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, APHP-Centre Université de Paris (CUP), Paris, France.

Uveitis can be associated with meningitis (uveomeningitis) and the inflammation shared with the central nervous system. We aimed to describe the characteristics and outcome of uveomeningitis. We retrospectively analyzed 110 consecutive adult patients with uveomeningitis. The main causes of uveomeningitis were Vogt-Koyanagi-Harada (31%), syphilis (16%), sarcoidosis (12%), Behçet's disease (7%), and multiple sclerosis (5%). Sixteen percent of uveomeningitis remained of undetermined origin. Compared to etiology-matched uveitis without meningitis, patients with uveomeningitis were younger, had more frequent neurological manifestations, and had more frequent abnormal cerebral magnetic resonance imaging findings. In contrast, no ocular feature upon examination was significantly associated with the presence of meningitis. Patients with uveomeningitis were more frequently treated with immunosuppressants but uveitis relapse and systemic complications did not differ between groups. Uveomeningitis is associated with a limited spectrum of diseases. Meningitis does not seem to impact ocular and extraocular outcomes. Therefore, lumbar puncture should be performed on an individual basis during the diagnostic workup of uveitis.
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http://dx.doi.org/10.1080/09273948.2021.1898000DOI Listing
May 2021

Primary biliary cholangitis and systemic sclerosis (Reynolds syndrome): A case-control study.

Autoimmun Rev 2021 Jul 7;20(7):102842. Epub 2021 May 7.

Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), France; APHP-CUP, Hôpital Cochin, Université de Paris, F-75014 Paris, France.

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http://dx.doi.org/10.1016/j.autrev.2021.102842DOI Listing
July 2021

Effects of non-pharmacological and non-surgical interventions on health outcomes in systemic sclerosis: protocol for a living systematic review.

BMJ Open 2021 05 4;11(5):e047428. Epub 2021 May 4.

Lady Davis Institute of the Jewish General Hospital, Montreal, Quebec, Canada

Introduction: Systemic sclerosis (SSc; scleroderma) is a rare, chronic, autoimmune disease with a high level of burden, a significant impact on the ability to carry out daily activities, and a considerable negative impact on health-related quality of life. Non-pharmacological interventions could be provided to potentially improve mental and physical health outcomes. However, the effectiveness of non-pharmacological interventions on health and well-being among individuals with SSc has not been well established. The proposed living systematic review aims to identify and evaluate randomised controlled trial (RCT) evidence on the effectiveness of non-pharmacological and non-surgical interventions on mental and physical health outcomes and on the delivery of such services in SSc.

Methods And Analysis: Eligible studies will be RCTs that examine non-pharmacological and non-surgical interventions aimed at improving health outcomes among individuals with SSc or the delivery of services intended to improve healthcare or support of people with SSc (eg, support groups). All RCTs included in a previous systematic review that sought studies published between 1990 and March 2014 will be evaluated for inclusion. Additional trials will be sought from January 2014 onwards using a similar, augmented search strategy developed by a health sciences librarian. We will search the MEDLINE, Embase, CINAHL, PsycINFO, Cochrane Library and Web of Science databases and will not restrict by language. Two independent reviewers will determine the eligibility of identified RCTs and will extract data using a prespecified standardised form in DistillerSR. Meta-analyses will be considered if ≥2 eligible RCTs report similar non-pharmacological interventions and comparable health outcomes. We will conduct a qualitative synthesis for interventions that cannot be synthesised via meta-analysis.

Ethics And Dissemination: We will post initial and ongoing results via a website, publish results periodically via peer-reviewed journal publication, and present results at patient-oriented events.

Prospero Registration Number: CRD42020219914.
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http://dx.doi.org/10.1136/bmjopen-2020-047428DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8098987PMC
May 2021

Evaluation of the severe preeclampsia classification criterion for antiphospholipid syndrome in a study of 40 patients.

Arthritis Res Ther 2021 05 4;23(1):134. Epub 2021 May 4.

AP-HP, Cochin Hospital, Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares d'Ile de France, Paris, France.

Background: The criteria for antiphospholipid syndrome (APS) include severe preeclampsia and/or placental insufficiency leading to preterm delivery before 34 weeks of gestation, but this APS manifestation has been rarely studied. Thus, we report a series of severe preeclampsia occurred in patients with APS.

Methods: We retrospectively analysed data of women with APS (Sydney criteria) who experienced severe preeclampsia with delivery before 34 weeks' gestation between 2000 and 2017 at five French internal medicine departments and one Italian rheumatology unit.

Results: The 40 women had a mean age of 30.5 ± 4.6 years at their first episode of preeclampsia; 21 were nulligravid (52.5%), 12 (30%) had already been diagnosed with APS, and 21 (52.5%) had a triple-positive antiphospholipid (aPL) antibody test. Preeclampsia occurred at a median gestational age of 25.5 weeks (IQR 23-29). It was associated with HELLP in 18 cases (45%), eclampsia in 6 (15%), placental abruption in 3 (7.5%), catastrophic APS in 3 (7.5%), and foetal and neonatal death in 11 and 15 cases. Overall, 14 (35%) children survived, born at a median gestational age of 31 weeks. Among other APS criteria, 16 women (40%) experienced at least one thrombosis, 17 (42.5%) an intrauterine foetal death, and 19 (47.5%) at least one episode of HELLP during follow-up (median 5 years, IQR = 2-8). None had three or more consecutive miscarriages. Notably, 12 women (30%) had systemic lupus erythematosus.

Conclusions: Severe preeclampsia led to high mortality in the offspring. Almost half of these women experienced other APS features, but not three consecutive miscarriages.
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http://dx.doi.org/10.1186/s13075-021-02518-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8094564PMC
May 2021

A French cohort of patients with giant cell arteritis: glucocorticoid treatment and its associated side effects.

Clin Exp Rheumatol 2021 Mar-Apr;39 Suppl 129(2):155-160. Epub 2021 Apr 30.

Department of Internal Medicine, Hôpital Cochin, Université de Paris, Sorbonne Paris Cité, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, Paris, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Paris, France.

Objectives: Giant cell arteritis (GCA) is the most common primary large-vessel vasculitis. Glucocorticoids (GC) therapy remains the standard of care for GCA despite frequent side effects (SEs). However, treatment modality changes, prophylactic treatment of osteoporosis, or vaccinations might have decreased the frequency of GC-related SEs. This study aims to describe GCA treatment and GC-related SEs in a recent cohort.

Methods: Patients with a diagnosis of GCA between May 2009 and March 2018 were included in this multicentric retrospective study. Characteristics of patients, treatment modalities and GC-related SEs were collected and analysed. Risk factors associated with the occurrence of SE were studied.

Results: We analysed the files from 206 patients (153 women, 53 men; median age 74 years). Median follow-up was 34 months. Patients received GC for a median of 25 months, starting at 0.7 mg/kg/day, with tapering to 5 mg/day after 11 months follow-up. Flares occurred in 83/201 (41%) patients. Among the 132 patients who stopped GC, 29 (22%) experienced a relapse. SEs occurred in 129 (64%) patients: bone fractures and infections in 13% each and hypertension onset in 9%. Age >75 years, treatment duration >2 years, past medical history of diabetes were risk factors associated with GC-related SEs.

Conclusions: Flares occur in 41% of patients during GC withdrawal. As much as 64% of patients had treatment related SEs. An age> 75 year and a past medical history of diabetes were predictive of SEs during follow-up.
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May 2021

Deep Learning-based Approach for Automated Assessment of Interstitial Lung Disease in Systemic Sclerosis on CT Images.

Radiol Artif Intell 2020 Jul 15;2(4):e190006. Epub 2020 Jul 15.

Departments of Radiology (G.C., N.J., M.P.R.) and Physiology (T.H.H., A.T.D.X.), Hôpital Cochin, and Reference Center for Rare Systemic Autoimmune Diseases of Ile de France, Hôpital Cochin (A.R., N. Benmostefa, L.M.), Assistance Publique-Hôpitaux de Paris, Université de Paris, 27 Rue du Faubourg Saint-Jacques, 75014 Paris, France; Center for Visual Computing, Ecole CentraleSupelec, Gif-sur-Yvette, France (G.C., M.V., E.I.Z., C.M., N.P.); Department of Radiology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel (G.A.); TheraPanacea, Paris, France (R.M., N. Bus, N.P.); and Departments of Internal Medicine and Inflammatory Disorders (A.M.) and Radiology (L.M.C.), Hôpital Saint-Antoine, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France.

Purpose: To develop a deep learning algorithm for the automatic assessment of the extent of systemic sclerosis (SSc)-related interstitial lung disease (ILD) on chest CT images.

Materials And Methods: This retrospective study included 208 patients with SSc (median age, 57 years; 167 women) evaluated between January 2009 and October 2017. A multicomponent deep neural network (AtlasNet) was trained on 6888 fully annotated CT images (80% for training and 20% for validation) from 17 patients with no, mild, or severe lung disease. The model was tested on a dataset of 400 images from another 20 patients, independently partially annotated by three radiologist readers. The ILD contours from the three readers and the deep learning neural network were compared by using the Dice similarity coefficient (DSC). The correlation between disease extent obtained from the deep learning algorithm and that obtained by using pulmonary function tests (PFTs) was then evaluated in the remaining 171 patients and in an external validation dataset of 31 patients based on the analysis of all slices of the chest CT scan. The Spearman rank correlation coefficient (ρ) was calculated to evaluate the correlation between disease extent and PFT results.

Results: The median DSCs between the readers and the deep learning ILD contours ranged from 0.74 to 0.75, whereas the median DSCs between contours from radiologists ranged from 0.68 to 0.71. The disease extent obtained from the algorithm, by analyzing the whole CT scan, correlated with the diffusion lung capacity for carbon monoxide, total lung capacity, and forced vital capacity (ρ -0.76, -0.70, and -0.62, respectively; < .001 for all) in the dataset for the correlation with PFT results. The disease extents correlated with diffusion lung capacity for carbon monoxide, total lung capacity, and forced vital capacity were ρ = -0.65, -0.70, and -0.57, respectively, in the external validation dataset ( < .001 for all).

Conclusion: The developed algorithm performed similarly to radiologists for disease-extent contouring, which correlated with pulmonary function to assess CT images from patients with SSc-related ILD.© RSNA, 2020.
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http://dx.doi.org/10.1148/ryai.2020190006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8082359PMC
July 2020

Effects of a multi-faceted education and support programme on anxiety symptoms among people with systemic sclerosis and anxiety during COVID-19 (SPIN-CHAT): a two-arm parallel, partially nested, randomised, controlled trial.

Lancet Rheumatol 2021 Jun 16;3(6):e427-e437. Epub 2021 Apr 16.

Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, QC, Canada.

Background: No trials have tested multifaceted mental health interventions recommended by public health organisations during COVID-19. The objective of this trial was to evaluate the effect of the Scleroderma Patient-centered Intervention Network COVID-19 Home-isolation Activities Together (SPIN-CHAT) Program on anxiety symptoms and other mental health outcomes among people vulnerable during COVID-19 owing to a pre-existing medical condition.

Methods: The SPIN-CHAT Trial was a pragmatic, two-arm, parallel, partially nested, randomised, controlled trial (1:1 allocation to intervention or waitlist). Eligible participants with systemic sclerosis were recruited from the international SPIN COVID-19 Cohort. SPIN COVID-19 Cohort participants were eligible for the trial if they completed baseline measures and had at least mild anxiety symptoms, had not tested positive for COVID-19, and were not currently receiving mental health counselling. SPIN-CHAT is a 4-week (3 sessions per week) videoconference-based group intervention that provided education and practice with mental health coping strategies, and provided social support to reduce isolation. Groups included 6-10 participants. The primary outcome analysed in the intention-to-treat population was anxiety symptoms (PROMIS Anxiety 4a version 1.0) immediately post-intervention. This trial is registered with ClinicalTrials.gov, NCT04335279 and is complete.

Findings: Of participants who completed baseline measures between April 9, 2020, and April 27, 2020, 560 participants were eligible and 172 participants were randomly assigned to intervention (n=86) or waitlist (n=86). Mean age was 55·0 years (SD 11·4 years), 162 (94%) were women, and 136 (79%) identified as White. In intention-to-treat analyses, the intervention did not significantly reduce anxiety symptoms post-intervention (-1·57 points, 95% CI -3·59 to 0·45; standardised mean difference [SMD] -0·22 points) but reduced symptoms 6 weeks later (-2·36 points, 95% CI -4·56 to -0·16; SMD -0·31). Depression symptoms were significantly lower 6 weeks post-intervention (-1·64 points, 95% CI -2·91 to -0·37; SMD -0·31); no other secondary outcomes were significant. No adverse events were reported.

Interpretation: The intervention did not significantly improve anxiety symptoms or other mental health outcomes post-intervention. However, anxiety and depression symptoms were significantly lower 6 weeks later, potentially capturing the time it took for new skills and social support between intervention participants to affect mental health. Multi-faceted interventions such as SPIN-CHAT have potential to address mental health needs in vulnerable groups during COVID-19, yet uncertainty remains about effectiveness.

Funding: Canadian Institutes of Health Research (CIHR; VR4-172745, MS1-173066); McGill Interdisciplinary Initiative in Infection and Immunity Emergency COVID-19 Research Fund; Scleroderma Canada, made possible by an educational grant for patient support programming from Boehringer Ingelheim; the Scleroderma Society of Ontario; Scleroderma Manitoba; Scleroderma Atlantic; Scleroderma Australia; Scleroderma New South Wales; Scleroderma Victoria; Scleroderma Queensland; Scleroderma SASK; the Scleroderma Association of BC; and Sclérodermie Québec.
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http://dx.doi.org/10.1016/S2665-9913(21)00060-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8051930PMC
June 2021

Myeloproliferative neoplasms and clonal hematopoiesis in patients with giant cell arteritis: a case-control and exploratory study.

Rheumatology (Oxford) 2021 Apr 9. Epub 2021 Apr 9.

Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune Diseases, Paris University, Paris, France.

Objectives: Giant cell arteritis (GCA) is a large vessel vasculitis for which triggering factors remain unknown. Clonal hematopoiesis (CH) was associated with atherosclerosis through the induction of inflammation in myeloid cells, and data suggest that CH expansion and inflammation may support each other to induce a proinflammatory loop. Our objective was to describe the impact of JAK2p.V617F-mutated myeloproliferative neoplasms (MPN) on GCA and to screen MPN-free patients for CH mutations.

Methods: We performed a retrospective case-control study comparing characteristics of 21 GCA patients with MPN and 42 age and gender-matched GCA patients without MPN. Also, 18 GCA patients were screened for CH through Next Generation Sequencing.

Results: The most frequent associated MPN was essential thrombocythemia (ET) (n = 11). Compared to controls, GCA patients with MPN had less frequent cephalic symptoms (71.4 vs. 97.6%, p = 0.004) and higher platelets count at baseline [485 (346-586) vs. 346 [IQR 296-418] x 109/L, p = 0.02). There was no difference between groups for other clinical features. Overall survival was significantly shorter in patients with MPN compared to controls [HR 8.2 (95% CI 1.2-56.6), p = 0.03]. Finally, screening for CH using NGS in 15 GCA patients without MPN revealed CH in 33%.

Conclusion: GCA patients with MPN display higher platelets count and shorter overall survival than controls. This association could not be fortuitous given the possible pathophysiological relationship between the two diseases. CH was found in one third of GCA patients, which may be higher than the expected prevalence for similar age, what should be confirmed in a larger cohort.
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http://dx.doi.org/10.1093/rheumatology/keab337DOI Listing
April 2021

An immunohistochemical analysis of fibroblasts in giant cell arteritis.

Ann Diagn Pathol 2021 Jun 1;52:151728. Epub 2021 Mar 1.

Department of Internal Medicine, Dupuytren Hospital, Limoges, France; EA3842-CaPTuR, Contrôle de l'Activation Cellulaire, Progression Tumorale et Résistance thérapeutique, Faculty of Medicine, Limoges, France.

Background: Giant cell arteritis (GCA) is a systemic vasculitis of large and medium vessels characterized by an inflammatory arterial infiltrate. GCA begins in the adventitia and leads to vascular remodeling by promoting proliferation of myofibroblasts in the intima. The morphology of the fibroblasts in the adventitia in GCA is unclear. Access to temporal artery biopsies allows morphological studies and evaluation of the microenvironment of the arterial wall. We evaluated the distribution of vascular fibroblasts and of markers of their activation in GCA.

Methods: Formalin-fixed paraffin-embedded tissue sections from 29 patients with GCA and 36 controls were examined. Immunohistochemistry was performed for CD90, vimentin, desmin, alpha-smooth muscle actin (ASMA), prolyl-4-hydroxylase (P4H), and myosin to evaluate the distribution of fibroblasts within the intima, media, and adventitia.

Results: Temporal arteries from patients with GCA showed increased levels of CD90, vimentin, and ASMA in the adventitia and intima compared to the controls. Desmin was expressed only in the media in both groups. P4H was expressed similarly in the adventitia and intima in the two groups. Adventitial and intimal CD90 cells co-expressed P4H, ASMA, and myosin at a high level in GCA.

Conclusion: The results suggest a role for adventitial fibroblasts in GCA. Inhibiting the differentiation of adventitial fibroblasts to myofibroblasts has therapeutic potential for GCA.
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http://dx.doi.org/10.1016/j.anndiagpath.2021.151728DOI Listing
June 2021

Metabolomic analyses of COVID-19 patients unravel stage-dependent and prognostic biomarkers.

Cell Death Dis 2021 03 11;12(3):258. Epub 2021 Mar 11.

Département d'Oncologie Médicale, Gustave Roussy Cancer Campus, 94800, Villejuif, France.

The circulating metabolome provides a snapshot of the physiological state of the organism responding to pathogenic challenges. Here we report alterations in the plasma metabolome reflecting the clinical presentation of COVID-19 patients with mild (ambulatory) diseases, moderate disease (radiologically confirmed pneumonitis, hospitalization and oxygen therapy), and critical disease (in intensive care). This analysis revealed major disease- and stage-associated shifts in the metabolome, meaning that at least 77 metabolites including amino acids, lipids, polyamines and sugars, as well as their derivatives, were altered in critical COVID-19 patient's plasma as compared to mild COVID-19 patients. Among a uniformly moderate cohort of patients who received tocilizumab, only 10 metabolites were different among individuals with a favorable evolution as compared to those who required transfer into the intensive care unit. The elevation of one single metabolite, anthranilic acid, had a poor prognostic value, correlating with the maintenance of high interleukin-10 and -18 levels. Given that products of the kynurenine pathway including anthranilic acid have immunosuppressive properties, we speculate on the therapeutic utility to inhibit the rate-limiting enzymes of this pathway including indoleamine 2,3-dioxygenase and tryptophan 2,3-dioxygenase.
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http://dx.doi.org/10.1038/s41419-021-03540-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7948172PMC
March 2021

Granulomatosis with polyangiitis: Study of 795 patients from the French Vasculitis Study Group registry.

Semin Arthritis Rheum 2021 04 10;51(2):339-346. Epub 2021 Feb 10.

National Referral Center for Rare Systemic Autoimmune Diseases, Université Paris Descartes, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), 27, rue du faubourg Saint-Jacques, Paris, Cedex 14 75679, France. Electronic address:

Objective: To describe the characteristics and long-term outcomes of patients with granulomatosis with polyangiitis (GPA) from the French Vasculitis Study Group database.

Methods: Patients' clinical and laboratory characteristics, Birmingham Vasculitis Activity Score (BVAS)-assessed disease activity, malignancies, opportunistic infections, and vital status were collected at diagnosis and each visit. Estimated probabilities and predictors of overall (OS) and relapse-free survival (RFS) were analyzed by Cox regression.

Results: We enrolled 795 newly diagnosed patients, followed for a median of 3.5 years. Initial clinical manifestations involved ear, nose & throat (ENT; 80%), lungs (68%) and kidneys (56%). Among the 728 available ELISA results, 75.0% were PR3-ANCA-positive, 16.5% MPO-ANCA-positive and 62 (8.5%) ANCA-negative. Relapses occurred in 394 (50%) patients, involving ≥1 organ(s) affected at onset in 179 (46%), mainly ENT, lungs and kidneys, with mean BVAS 10.2 points below that at diagnosis (p<0.001). Five- and 10-year RFS rates were 37% and 17%, respectively. PR3-ANCA-positivity independently predicted relapse (p = 0.05) and prolonged survival (p = 0.038). OS-but not RFS-improved significantly over time (p<0.001); 10-year OS reached 88.2% (95% CI 83.9 to 92.7) for the 660 patients diagnosed after 2000. Infections were the main causes of death. Malignancy or opportunistic infection each occurred in ≤5% of the patients.

Conclusion: Survival has improved dramatically over the last decades but the high relapse rate remains a major concern for GPA patients, once again stressing the need for therapeutic strategy optimization to lower it. PR3-ANCA-positivity was associated with increased probability of relapse and survival.
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http://dx.doi.org/10.1016/j.semarthrit.2021.02.002DOI Listing
April 2021

Barriers and facilitators to physical activity for people with scleroderma: a Scleroderma Patient-centered Intervention Network (SPIN) Cohort study.

Arthritis Care Res (Hoboken) 2021 Feb 2. Epub 2021 Feb 2.

Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Canada, Quebec.

Objective: To support physical activity among people with systemic sclerosis (SSc; scleroderma), we sought to determine the (1) prevalence and importance of barriers and (2) likelihood of using possible facilitators.

Methods: We invited 1,707 participants from an international SSc cohort to rate the (1) importance of 20 barriers (14 medical; 4 social or personal; 1 lifestyle; 1 environmental); and (2) likelihood of using 91 corresponding barrier-specific and 12 general facilitators.

Results: Among 721 respondents, 13 barriers were experienced by ≥25% of participants, including 2 (fatigue, Raynaud's) rated 'important' or 'very important' by ≥50% of participants, 7 (joint stiffness and contractures, shortness of breath, gastrointestinal problems, difficulty grasping, pain, muscle weakness and mobility limitations, low motivation) by 26-50%, and 4 by <26%. Overall, 23 (18 medical-related) of 103 facilitators were rated by ≥75% as 'likely' or 'very likely' to use among those who experienced corresponding barriers; these facilitators focused on adapting exercise (e.g., using controlled, slow movement), taking care of one's body (e.g., stretching), keeping warm (e.g., wearing gloves), and protecting skin (e.g., covering ulcers). Among those who had previously tried the facilitator, all facilitators were rated by ≥50% as 'likely' or 'very likely' to use; among those with the barrier who had not tried the facilitator, only 12 of 103 were rated by >50% as 'likely' or 'very likely'.

Conclusion: Medical-related physical activity barriers were common and considered important. Facilitators considered as most likely to be used involved adapting exercise, taking care of one's body, keeping warm, and protecting skin.
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http://dx.doi.org/10.1002/acr.24567DOI Listing
February 2021

The impact of COVID-19 on rare and complex connective tissue diseases: the experience of ERN ReCONNET.

Nat Rev Rheumatol 2021 03 6;17(3):177-184. Epub 2021 Jan 6.

Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium.

During the COVID-19 pandemic, the need to provide high-level care for a large number of patients with COVID-19 has affected resourcing for, and limited the routine care of, all other conditions. The impact of this health emergency is particularly relevant in the rare connective tissue diseases (rCTDs) communities, as discussed in this Perspective article by the multi-stakeholder European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET). The clinical, organizational and health economic challenges faced by health-care providers, institutions, patients and their families during the SARS-CoV-2 outbreak have demonstrated the importance of ensuring continuity of care in the management of rCTDs, including adequate diagnostics and monitoring protocols, and highlighted the need for a structured emergency strategy. The vulnerability of patients with rCTDs needs to be taken into account when planning future health policies, in preparation for not only the post-COVID era, but also any possible new health emergencies.
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http://dx.doi.org/10.1038/s41584-020-00565-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7786339PMC
March 2021

Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

Rheumatology (Oxford) 2020 Dec 21. Epub 2020 Dec 21.

Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune Diseases, Paris University, Paris, France.

Objectives: Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA.

Methods: We set up a retrospective European multicentre cohort including 845 patients. Baseline characteristics and outcomes were analysed and compared according to ANCA status.

Results: ANCA status was available for 734 patients: 508 (69.2%) ANCA-negative, 210 (28.6%) MPO-ANCA and 16 (2.2%) PR3-ANCA. At baseline, PR3-ANCA patients, compared with those with MPO-ANCA and ANCA-negative, less frequently had active asthma (69% vs 91% and 93%, P = 0.003, respectively) and peripheral neuropathy (31% vs 71% and 47%, P < 0.0001), more frequently had cutaneous manifestations (63% vs 38% and 34%, P = 0.03) and pulmonary nodules (25% vs 10% and 8%, P = 0.046), and lower median eosinophil count (1450 vs 5400 and 3224/mm3, P < 0.0001). Vasculitis relapse-free survival was shorter for PR3-ANCA (hazard ratio 6.05, P = 0.005) and MPO-ANCA patients (hazard ratio 1.88, P = 0.0002) compared with ANCA-negative patients.

Conclusion: PR3-ANCA EGPA patients differ from those with MPO-ANCA and negative ANCA, and share clinical features with granulomatosis with polyangiitis. This suggests that PR3-ANCA EGPA could be a particular form of PR3-ANCA-associated vasculitis.
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http://dx.doi.org/10.1093/rheumatology/keaa805DOI Listing
December 2020

Pre-test probability for SARS-Cov-2-related infection score: The PARIS score.

PLoS One 2020 17;15(12):e0243342. Epub 2020 Dec 17.

Department of Radiology, Cochin Hospital, APHP, Paris, France.

Introduction: In numerous countries, large population testing is impossible due to the limited availability of RT-PCR kits and CT-scans. This study aimed to determine a pre-test probability score for SARS-CoV-2 infection.

Methods: This multicenter retrospective study (4 University Hospitals) included patients with clinical suspicion of SARS-CoV-2 infection. Demographic characteristics, clinical symptoms, and results of blood tests (complete white blood cell count, serum electrolytes and CRP) were collected. A pre-test probability score was derived from univariate analyses of clinical and biological variables between patients and controls, followed by multivariate binary logistic analysis to determine the independent variables associated with SARS-CoV-2 infection.

Results: 605 patients were included between March 10th and April 30th, 2020 (200 patients for the training cohort, 405 consecutive patients for the validation cohort). In the multivariate analysis, lymphocyte (<1.3 G/L), eosinophil (<0.06 G/L), basophil (<0.04 G/L) and neutrophil counts (<5 G/L) were associated with high probability of SARS-CoV-2 infection but no clinical variable was statistically significant. The score had a good performance in the validation cohort (AUC = 0.918 (CI: [0.891-0.946]; STD = 0.014) with a Positive Predictive Value of high-probability score of 93% (95%CI: [0.89-0.96]). Furthermore, a low-probability score excluded SARS-CoV-2 infection with a Negative Predictive Value of 98% (95%CI: [0.93-0.99]). The performance of the score was stable even during the last period of the study (15-30th April) with more controls than infected patients.

Conclusions: The PARIS score has a good performance to categorize the pre-test probability of SARS-CoV-2 infection based on complete white blood cell count. It could help clinicians adapt testing and for rapid triage of patients before test results.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0243342PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7745977PMC
December 2020

Osteitis in Systemic Sclerosis: a nationwide case-control retrospective study (SCLEROS Study).

Arthritis Care Res (Hoboken) 2020 Dec 5. Epub 2020 Dec 5.

Service de Médecine Interne, Centre de Référence Maladies systémiques auto-immunes rares d'Ile de France, Hôpital Cochin, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.

Objective: Systemic sclerosis (SSc) is an autoimmune (AI) connective tissue disorder characterized by skin fibrosis, vasculopathy and dysimmunity. Data regarding osteitis in SSc are scarce.

Method: We performed a nationwide multicentre retrospective case-control study including patients with SSc according to the 2013 ACR/EULAR classification, with a diagnosis of osteitis. The objectives of the study were to describe, to characterize, and to identify associated factors for osteitis in patients with SSc.

Results: Forty-eight patients were included. Twenty-six patients (54.1%) had osteitis beneath digital tip ulcers. Physical symptoms included: pain (36/48, 75%), erythema (35/48, 73%), and local warmth (35/48, 73%). Thirty-one (65%) patients had C-reactive protein levels >2 mg/L (8 [2.7 - 44.3] mg/L). On X-ray, CT-scans or MRI, osteitis was characterized by swelling or abscess of soft tissues with acro-osteolysis or lysis in 28 patients (58%). Microbiological sampling was performed in 45 (94%) patients. Most pathogens were Staphylococcus aureus (43.8%); anaerobes and Enterobacteriaceae (29.1%) and Pseudomonas aeruginosa (10.4%). Management comprised antibiotics in 37 (77.1%) patients and/or surgery in 26 (54.2%). Fluoroquinolones were used in 22 (45.8%) patients and amoxicillin + beta-lactamase inhibitor in 7 (14.6%). Six (12.6%) patients relapsed, 6 (12.6%) patients had osteitis recurrence, 15 (32%) sequelae, and 2 patients had septic shock and died.

Conclusion: This study confirmed digital tip ulcers as an associated factor for osteitis, and revealed a high rate of functional sequelae. Antimicrobial therapy with oral fluoroquinolone or intravenous amoxicillin and beta-lactamase inhibitor are used as first-line antibiotherapy in SSc patients with osteitis.
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http://dx.doi.org/10.1002/acr.24530DOI Listing
December 2020

Factors associated with fears due to COVID-19: A Scleroderma Patient-centered Intervention Network (SPIN) COVID-19 cohort study.

J Psychosom Res 2021 01 25;140:110314. Epub 2020 Nov 25.

Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics, and Occupational Health, McGill University, Montreal, Quebec, Canada; Department of Medicine, McGill University, Montreal, Quebec, Canada; Department of Psychology, McGill University, Montreal, Quebec, Canada; Department of Educational and Counselling Psychology, McGill University, Montreal, Quebec, Canada; Biomedical Ethics Unit, McGill University, Montreal, Quebec, Canada. Electronic address:

Introduction: No studies have examined factors associated with fear in any group of people vulnerable during COVID-19 due to pre-existing medical conditions.

Objective: To investigate factors associated with fear of consequences of COVID-19 among people living with a pre-existing medical condition, the autoimmune disease systemic sclerosis (SSc; scleroderma), including country.

Methods: Pre-COVID-19 data from the Scleroderma Patient-centered Intervention Network (SPIN) Cohort were linked to COVID-19 data collected in April 2020. Multivariable linear regression was used to assess factors associated with continuous scores of the 10-item COVID-19 Fears Questionnaire for Chronic Medical Conditions, controlling for pre-COVID-19 anxiety symptoms.

Results: Compared to France (N = 156), COVID-19 Fear scores among participants from the United Kingdom (N = 50) were 0.12 SD (95% CI 0.03 to 0.21) higher; scores for Canada (N = 97) and the United States (N = 128) were higher, but not statistically significant. Greater interference of breathing problems was associated with higher fears due to COVID-19 (Standardized regression coefficient = 0.12, 95% CI 0.01 to 0.23). Participants with higher financial resources adequacy scores had lower COVID-19 Fear scores (Standardized coefficient = -0.18, 95% CI -0.28 to -0.09).

Conclusions: Fears due to COVID-19 were associated with clinical and functional vulnerabilities in this chronically ill population. This suggests that interventions may benefit from addressing specific clinical issues that apply to specific populations. Financial resources, health policies and political influences may also be important. The needs of people living with chronic illness during a pandemic may differ depending on the social and political context in which they live.
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http://dx.doi.org/10.1016/j.jpsychores.2020.110314DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7685938PMC
January 2021

Role of therapeutic plasma exchanges in systemic vasculitis.

Transfus Apher Sci 2020 Dec 2;59(6):102992. Epub 2020 Nov 2.

Service de Médecine interne, Centre de Référence Maladies auto-immunes et systémiques rares d'Ile de France, Hôpital Cochin, Assistance Publique Hôpitaux de Paris (AP-HP), 27 rue du Faubourg Saint Jacques, Paris, France; Université de Paris, INSERM U970, PARCC, F-75015, Paris, France.

Therapeutic management of systemic vasculitides is based on glucocorticoids (GCs), in combination or not with immunosuppressive and/or immunomodulatory agents. Plasma exchange (PLEX) has been used in some vasculitides, with various levels of evidence. Indeed, it is part of the first-line therapy in patients with anti-glomerular basement membrane (GBM) antibodies and in patients with severe hepatitis B related polyarteritis nodosa (PAN). PLEX might also be considered in some selected patients with ANCA-associated vasculitis and cryoglobulinemia vasculitis. Studies support the use of PLEX as second line therapy in refractory Kawasaki disease. Finally, there is not robust data to support the use of PLEX in unselected patients with non-HBV-related PAN or IgA vasculitis. Additional studies are required to address its role in these settings.
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http://dx.doi.org/10.1016/j.transci.2020.102992DOI Listing
December 2020

Validation of the COVID-19 Fears Questionnaires for Chronic Medical Conditions: A Scleroderma Patient-centered Intervention Network COVID-19 Cohort study.

J Psychosom Res 2020 12 8;139:110271. Epub 2020 Oct 8.

Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada; Department of Psychiatry, McGill University, Montreal, Quebec, Canada; Department of Epidemiology, Biostatistics and Occupational Health, McGill University, Montreal, Quebec, Canada; Department of Psychology, McGill University, Montreal, Quebec, Canada; Department of Educational and Counselling Psychology, McGill University, Montreal, Quebec, Canada; Biomedical Ethics Unit, McGill University, Montreal, Quebec, Canada. Electronic address:

Objective: Fear associated with medical vulnerability should be considered when assessing mental health among individuals with chronic medical conditions during the COVID-19 pandemic. The objective was to develop and validate the COVID-19 Fears Questionnaire for Chronic Medical Conditions.

Methods: Fifteen initial items were generated based on suggestions from 121 people with the chronic autoimmune disease systemic sclerosis (SSc; scleroderma). Patients in a COVID-19 SSc cohort completed items between April 9 and 27, 2020. Exploratory factor analysis (EFA) and item analysis were used to select items for inclusion. Cronbach's alpha and Pearson correlations were used to evaluate internal consistency reliability and convergent validity. Factor structure was confirmed with confirmatory factor analysis (CFA) in follow-up data collection two weeks later.

Results: 787 participants completed baseline measures; 563 of them completed the follow-up assessment. Ten of 15 initial items were included in the final questionnaire. EFA suggested that a single dimension explained the data reasonably well. There were no indications of floor or ceiling effects. Cronbach's alpha was 0.91. Correlations between the COVID-19 Fears Questionnaire and measures of anxiety (r = 0.53), depressive symptoms (r = 0.44), and perceived stress (r = 0.50) supported construct validity. CFA supported the single-factor structure (χ(35) = 311.2, p < 0.001, Tucker-Lewis Index = 0.97, Comparative Fit Index = 0.96, Root Mean Square Error of Approximation = 0.12).

Conclusion: The COVID-19 Fears Questionnaire for Chronic Medical Conditions can be used to assess fear among people at risk due to pre-existing medical conditions during the COVID-19 pandemic.
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http://dx.doi.org/10.1016/j.jpsychores.2020.110271DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7543945PMC
December 2020

Elastic Registration-driven Deep Learning for Longitudinal Assessment of Systemic Sclerosis Interstitial Lung Disease at CT.

Radiology 2021 01 20;298(1):189-198. Epub 2020 Oct 20.

From the Department of Radiology (G.C., T.N.H.T., M.P.R.), Department of Internal Medicine, Reference Center for Rare Systemic Autoimmune Diseases of Île de France (A.R., B.D., L.M.), and Department of Physiology (A.T.D.X.), Hôpital Cochin, AP-HP Centre, Université de Paris, 27 Rue du Faubourg Saint-Jacques, 75014 Paris, France; Center for Visual Computing, École CentraleSupélec, Png-sur-Yvette, France (G.C., M.V., M.S., N.P.); and TheraPanacea, Paris, France (R.M., N.P.).

Background Longitudinal follow-up of interstitial lung diseases (ILDs) at CT mainly relies on the evaluation of the extent of ILD, without accounting for lung shrinkage. Purpose To develop a deep learning-based method to depict worsening of ILD based on lung shrinkage detection from elastic registration of chest CT scans in patients with systemic sclerosis (SSc). Materials and Methods Patients with SSc evaluated between January 2009 and October 2017 who had undergone at least two unenhanced supine CT scans of the chest and pulmonary function tests (PFTs) performed within 3 months were retrospectively included. Morphologic changes on CT scans were visually assessed by two observers and categorized as showing improvement, stability, or worsening of ILD. Elastic registration between baseline and follow-up CT images was performed to obtain deformation maps of the whole lung. Jacobian determinants calculated from the deformation maps were given as input to a deep learning-based classifier to depict morphologic and functional worsening. For this purpose, the set was randomly split into training, validation, and test sets. Correlations between mean Jacobian values and changes in PFT measurements were evaluated with the Spearman correlation. Results A total of 212 patients (median age, 53 years; interquartile range, 45-62 years; 177 women) were included as follows: 138 for the training set (65%), 34 for the validation set (16%), and 40 for the test set (21%). Jacobian maps demonstrated lung parenchyma shrinkage of the posterior lung bases in patients found to have worsened ILD at visual assessment. The classifier detected morphologic and functional worsening with an accuracy of 80% (32 of 40 patients; 95% confidence interval [CI]: 64%, 91%) and 83% (33 of 40 patients; 95% CI: 67%, 93%), respectively. Jacobian values correlated with changes in forced vital capacity ( = -0.38; 95% CI: -0.25, -0.49; < .001) and diffusing capacity for carbon monoxide ( = -0.42; 95% CI: -0.27, -0.54; < .001). Conclusion Elastic registration of CT scans combined with a deep learning classifier aided in the diagnosis of morphologic and functional worsening of interstitial lung disease in patients with systemic sclerosis. © RSNA, 2020 See also the editorial by Verschakelen in this issue.
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http://dx.doi.org/10.1148/radiol.2020200319DOI Listing
January 2021
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