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Autoimmun Rev 2022 Jun 22:103133. Epub 2022 Jun 22.

Centre Hospitalier Universitaire, Medecine Interne, Toulouse, France; Centre Hospitalier Universitaire, Laboratoire d'Immunologie, Toulouse, France; Clinique Saint-Exupery, Medecine Interne, Toulouse, France; Centre Hospitalier Universitaire, Néphrologie, Toulouse, France; Centre Hospitalier Universitaire, Cardiologie, Toulouse, France; Clinique Ambroise-Paré, Medecine Interne, Toulouse, France; Centre Hospitalier Universitaire, Pneumologie, Toulouse, France; Hopital Joseph Ducuing, Medecine Interne, Toulouse, France; Centre D'investigation Clinique (CIC), 1436 PEPSS Team, Toulouse, France.

Introduction: An association of systemic sclerosis (SSc) with cryoglobulin and/or cryofibrinogenemia has been described. However, clinical, biological, morphological and prognostic implications are unknown. The objective of this study was to describe the phenotype and evaluate the prognosis of cryoglobulinemia and/or cryofibrinogenemia in the progression of SSc. Read More

J Dermatol 2022 Jun 25. Epub 2022 Jun 25.

Department of Dermatology, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan.

Systemic sclerosis (SSc) is an autoimmune disease characterized by skin and lung fibrosis. Over 90% of patients with SSc are positive for autoantibodies. In addition, the serum levels of B-cell activating factor, a potent B-cell stimulator, are correlated with SSc severity and activity. Read More

Rheumatology (Oxford) 2022 Jun 25. Epub 2022 Jun 25.

Department of Biomedical Data Science, Geisel School of Medicine at Dartmouth, Lebanon, NH, 03756, USA.

Objectives: Four intrinsic molecular subsets (Inflammatory, Fibroproliferative, Limited, Normal-like) have previously been identified in systemic sclerosis (SSc) and are characterized by unique gene expression signatures and pathways. The intrinsic subsets have been linked to improvement with specific therapies. Here, we investigated associations between baseline demographics and intrinsic molecular subsets in a meta-analysis of published datasets. Read More

Curr Opin Pulm Med 2022 Jul;28(4):321-336

Exeter Respiratory Institute University of Exeter, Exeter.

Purpose Of Review: Ageing, the accrual of molecular and cellular damage over a lifetime confers progressive physiologic dysfunction of bodily systems, leaving the body in a heightened state of vulnerability to biophysical and psychosocial stressors. The inflection point is frailty which easily leads to disability and death. Interstitial lung disease (ILD) creates biophysical and psychosocial stresses difficult for even optimally fit patients to cope with. Read More

Front Oncol 2022 7;12:854499. Epub 2022 Jun 7.

Department of Rheumatology & Immunology, Wexner Medical Center, The Ohio State University, Columbus, OH, United States.

A complex relationship exists between rheumatic diseases and cancer. This delicate balance between chronic inflammation and malignant cell transformation in hematologic neoplasms has been observed, but is not well defined. Large Granular Lymphocyte (LGL) leukemia is at the intersection of a clonal lymphoproliferative disease, chronic inflammation, and autoimmunity. Read More

J Clin Med 2022 Jun 7;11(12). Epub 2022 Jun 7.

Rheumatology Clinic, Internal Medicine Unit, AOE Cannizzaro, 95126 Catania, Italy.

(1) Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction and fibrosis of skin and visceral organs. In the last decade, attention has been focused on the macrovascular involvement of the disease. In particular, the observation of increased arterial stiffness represented an interesting aspect of the disease, as predictor of cardiovascular risk. Read More

Arthritis Res Ther 2022 Jun 23;24(1):151. Epub 2022 Jun 23.

Division of Clinical Epidemiology, McGill University Health Centre, Montreal, QC, Canada.

Objectives: To estimate associations between fine particulate matter (PM) and ozone and the onset of systemic autoimmune rheumatic diseases (SARDs).

Methods: An open cohort of over 6 million adults was constructed from provincial physician billing and hospitalization records between 2000 and 2013. We defined incident SARD cases (SLE, Sjogren's syndrome, scleroderma, polymyositis, dermatomyositis, polyarteritis nodosa and related conditions, polymyalgia rheumatic, other necrotizing vasculopathies, and undifferentiated connective tissue disease) based on at least two relevant billing diagnostic codes (within 2 years, with at least 1 billing from a rheumatologist), or at least one relevant hospitalization diagnostic code. Read More

Acta Clin Croat 2021 Dec;60(4):711-721

1Department of Dermatovenereology, Sestre milosrdnice University Hospital Center, Zagreb, Croatia; 2School of Dental Medicine, University of Zagreb, Zagreb, Croatia; 3Unit of Dermatology and Venereology, Zabok General Hospital, Bračak/Zabok, Croatia; 4Faculty of Dental Medicine and Health, Josip Juraj Strossmayer University of Osijek, Osijek, Croatia; 5Ljudevit Jurak Department of Pathology, Sestre milosrdnice University Hospital Center, Zagreb, Croatia.

Although there are many single case reports on paraneoplastic dermatoses in the literature, there are very rare articles containing multiple cases. A retrospective study was performed to examine paraneoplastic dermatoses and accompanying malignancies based on skin manifestations and appropriate diagnostic evaluations. We recorded outcomes, current conditions, and surgical/oncologic treatments. Read More

Front Med (Lausanne) 2022 6;9:859330. Epub 2022 Jun 6.

Department of Dermatology, Dermatology Hospital, Southern Medical University, Guangzhou, China.

Background: Systemic sclerosis (SSc) is a rare disabling connective tissue disease with few available treatment options. Diffuse cutaneous systemic sclerosis (dcSSc) is associated with high mortality. A previous experiment has shown that JAK2 inhibitor can significantly improve skin fibrosis in bleomycin (BLM)-induced murine model, including reducing dermal thickening and collagen accumulation. Read More

BMC Complement Med Ther 2022 Jun 22;22(1):167. Epub 2022 Jun 22.

Zhang Zhongjing College of Chinese Medicine, Nanyang Institute of Technology, Changjiang Road 80, Nanyang, 473004, Henan, China.

Background: Vascular damage, autoimmune abnormalities, and fibrosis are the three pathological features of systemic sclerosis (SSc).However, pulmonary vascular damage is the main factor affecting the progression and prognosis of SSc. The main purpose of this study was to explore the molecular mechanism of Wenyang Huazhuo Tongluo Formula in alleviating pulmonary vascular injury in bleomycin-induced SSc mouse model. Read More

Rheumatology (Oxford) 2022 Jun 22. Epub 2022 Jun 22.

Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, USA.

Objectives: The early trajectory of skin fibrosis provides insights into the disease course of systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. The aims of our study were to ascertain the prevalence and characteristics of late skin fibrosis in SSc.

Methods: We developed and tested three conceptual scenarios of late (>5 years after 1st non-RP feature) skin fibrosis including new worsening of skin disease, and failure to improve after worsening within 5-year window. Read More

Arthritis Res Ther 2022 Jun 21;24(1):148. Epub 2022 Jun 21.

Department of Dermatology, Zhongshan Hospital, Fudan University, Xuhui District, 180 Fenglin Road, Shanghai, 200032, People's Republic of China.

Background: Calpains are a family of calcium-dependent thiol proteases that participate in a wide variety of biological activities. In our recent study, calpain is increased in the sera of scleroderma or systemic sclerosis (SSc). However, the role of calpain in interstitial lung disease (ILD) has not been reported. Read More

J Med Case Rep 2022 Jun 21;16(1):258. Epub 2022 Jun 21.

Mohammed V University of Rabat, Rabat, Morocco.

Background: Systemic sclerosis is a multisystemic character autoimmune disease. It is characterized by vascular dysfunction and progressive fibrosis affecting mainly the skin but also different internal organs. All heart structures are commonly affected, including the pericardium, myocardium, and conduction system. Read More

Br J Pharmacol 2022 Jun 21. Epub 2022 Jun 21.

Immunology Department, Hospital Universitario de la Princesa, Fundación de Investigación Biomédica (FIB), Instituto de Investigación Sanitaria-Princesa (IIS-Princesa), Madrid, Spain.

Background And Purpose: Interstitial lung disease (ILD) is the main cause of mortality in systemic sclerosis (SSc) and current therapies available are of low efficacy or high toxicity. Thus, the identification of innovative less toxic and high efficacy therapeutic approaches to ILD treatment is a crucial point. P-selectin Glycoprotein Ligand-1 (PSGL-1) interaction with P-selectin initiates leukocyte extravasation and the lack of its expression brings to SSc-like syndrome with high incidence of ILD in aged mice. Read More

Front Oral Health 2022 20;3:903154. Epub 2022 May 20.

Division of Oral Medicine and Dentistry, Brigham and Women's Hospital and Dana Farber Cancer Institute Department of Oral Medicine, Infection and Immunity, Harvard School of Dental Medicine, Boston, MA, United States.

Chronic oral graft-versus-host disease (cGVHD) is a complex, frequent, and highly impactful complication of allogeneic hematopoietic cell transplantation (alloHCT). It represents the leading cause of morbidity and mortality in long-term alloHCT survivors. cGVHD can affect almost any visceral organ system and commonly affects the skin, eyes and mouth, manifesting with signs and symptoms similar to other known immune-mediated and autoimmune diseases. Read More

Med Clin (Barc) 2022 Jun 16. Epub 2022 Jun 16.

Departmento de Medicina Interna, Hospital Universitario San Cecilio, Granada, España.

Arthritis Res Ther 2022 Jun 18;24(1):146. Epub 2022 Jun 18.

Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 1830 E. Monument Street, Room 540, Baltimore, MD, 21205, USA.

Background: Echocardiography (2DE) is integral for screening and longitudinal evaluation of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). In the present study, we sought to establish the reliability, repeatability, and reproducibility of 2DE parameters in SSc patients with and without PAH and to define the minimal detectable difference (MDD), the smallest change detected beyond measurement error.

Methods: SSc patients without known PAH and with invasively confirmed PAH on stable therapies underwent 2DE with strain at two time points. Read More

J Am Acad Dermatol 2022 Jun 15. Epub 2022 Jun 15.

Rheumatol Ther 2022 Jun 18. Epub 2022 Jun 18.

Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Camino del Molino, 2. 28942, Fuenlabrada, Madrid, Spain.

Arthritis Rheumatol 2022 Jun 17. Epub 2022 Jun 17.

INSERM UMR1227 LBAI, Lymphocytes B, Autoimmunité et Immunothérapies, University of Brest, Brest, France.

Rheumatology (Oxford) 2022 Jun 17. Epub 2022 Jun 17.

Muscle Research Unit, Internal Medicine Service, Hospital Clínic de Barcelona (HCB, ), Universidad de Barcelona and Center for Biomedical Research on Rare Diseases (CIBERER), Barcelona, Spain.

Objectives: To analyze the clinico-serological and histological phenotype of patients with systemic sclerosis (SSc) with associated myopathy.

Methods: From November 2002 to September 2020, 52 patients with SSc underwent a muscle biopsy for suspected myopathy. We established two subgroups according to the histological findings based on the presence of isolated fibrosis or fibrosis together with significant inflammation. Read More

J Pak Med Assoc 2022 May;72(5):972-974

Department of Physiology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Supervised training exercises are the mainstay of pulmonary rehabilitation (PR) in patients with chronic lung diseases like interstitial lung disease (ILD). A 40 years old female patient with systemic sclerosis clinically presenting with usual interstitial pneumonia (UIP) and pulmonary hypertension underwent an eight-week supervised exercise training programme. Forced Vital Capacity (FVC) showed improvement from 48. Read More

Oman Med J 2022 May 31;37(3):e376. Epub 2022 May 31.

Rheumatology Unit, Department of Medicine, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman.

Mixed connective tissue disease (MCTD) is a unique disorder characterized by the presence of a high titer of anti-U1 ribonucloprotein antibody with overlapping features of various connective tissue disorders including systemic lupus erythematosus, scleroderma, and myositis. Severe renal or neurological involvement is unusual with this disorder. We describe a case of biopsy-confirmed severe sensorimotor axonal polyneuropathy in a middle-aged gentleman as his first presentation for MCTD. Read More

Front Med (Lausanne) 2022 31;9:941370. Epub 2022 May 31.

State Key Laboratory of Respiratory Disease, National Clinical Center for Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, China.

Rheumatology (Oxford) 2022 Jun 16. Epub 2022 Jun 16.

Deptartment of Experimental and Clinical Medicine & Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy.

Objectives: Lung vascular and parenchymal changes can be recently assessed quantitatively in thoracic computed tomography (CT) images using automated software tools. We investigated the vessel parameters of patients with Systemic Sclerosis (SSc), quantified by CT imaging, and correlated them with the interstitial lung disease (ILD) features.

Methods: SSc patients undergoing standard of care pulmonary function testing and CT evaluation were retrospectively evaluated. Read More

Curr Opin Rheumatol 2022 Jun 10. Epub 2022 Jun 10.

Division of Rheumatology, Department of Medicine.

Purpose Of Review: Raynaud's phenomenon (RP) is a common vasospastic condition that results in digital hypoperfusion in response to cold and/or emotional stress and is associated with significant pain and disability. The aim of our review is to provide a practical approach for clinicians to inform assessment and management of patients with RP.

Recent Findings: Autoantibodies and nailfold capillaroscopy are key investigations to stratify the risk of progression to systemic sclerosis (SSc) in patients RP, which was recently confirmed in the multicenter, very early diagnosis of systemic sclerosis (VEDOSS) project. Read More

Int J Rheum Dis 2022 Jun 14. Epub 2022 Jun 14.

Division of Rheumatology and School of Medicine, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA.

Objective: Many indigenous non-Caucasian populations, including Native Americans, have been reported to have higher rates, distinct clinical phenotypes, increased complications, and greater severity of systemic sclerosis (SSc). However, little is known of SSc specifically in Native Americans of the American Southwest. This study compared the clinical and serologic manifestations and outcomes of SSc in Native Americans and non-Native Americans (non-Natives) of this region. Read More

Arch Bronconeumol 2021 Sep;57(9):602-604

Pulmonology Department in Centro Hospitalar Universitário Lisboa Norte, EPE, Av. Prof. Egas Moniz, n/s, 1649/035 Lisboa, Portugal.

Cureus 2022 May 13;14(5):e24973. Epub 2022 May 13.

General Surgery, West Suburban Medical Center, Chicago, USA.

Mixed connective tissue disease (MCTD) is a rare autoimmune condition that shows an overlap of at least two connective tissue diseases (CTD) including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), scleroderma, Sjögren's syndrome, polymyositis, or dermatomyositis. From a laboratory standpoint, MCTD is associated with high titers of anti-U1-RNP antibodies, which makes it difficult to determine whether it is a variant of each of the respective CTDs or a different entity altogether. Our objective is to report the case of a patient with MCTD presenting with status epilepticus who was ultimately diagnosed with lupus cerebritis. Read More

Int J Med Sci 2022 9;19(5):867-877. Epub 2022 May 9.

Rheumatology Clinic - Department of Medical and Surgical Sciences, University of Foggia, Foggia- Italy.

IL-17 modulates the synthesis of several molecules involved in the pathogenesis of Systemic Sclerosis (SSc). Vitamin D (1,25(OH)D3) shows anti-fibrotic properties and it is able to affect the IL-17 production in several experimental conditions. The aim of this study is to assess the production of IL-17A and pro-fibrotic cytokines in peripheral blood mononuclear cells (PBMCs) from subjects with SSc in basal conditions and after treatment with 1,25(OH)2D3 and IL-17A neutralizing antibodies. Read More