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Presse Med 2020 Jul 4:104038. Epub 2020 Jul 4.

McMaster University, Hamilton, Ontario, Canada.

There have been great advances in the management of ANCA associated vasculitis over the past decades. We have gone from an era where the disease was almost universally fatal to trying to prevent long term side effects of treatment regimens. With the ability to use pulse cyclophosphamide or rituximab as alternates to oral cyclophosphamide for induction of remission, side effects of therapy have been greatly reduced. Read More

Eur Arch Otorhinolaryngol 2020 Jul 4. Epub 2020 Jul 4.

Royal National ENT and Eastman Dental Hospitals, 47-49 Huntley Street, London, WC1E 6DG, UK.

Purpose: To recommend an international multidisciplinary medical and surgical algorithm of treatment in nasal vasculitis, which will create a more streamlined approach.

Methods: A two-centre, international retrospective analysis of granulomatosis with polyangiitis (GPA) and levamisole-associated vasculitis (LAV) cases presenting between 2005 and 2019 was carried out. Demographic data, and surgical and medical treatment were recorded. Read More

Mod Rheumatol 2020 Jul 2:1-25. Epub 2020 Jul 2.

Department of Rheumatology, Faculty of Medicine, Saga University, Saga, Japan.

We examined the efficacy and safety of rituximab (RTX) maintenance therapy for patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan. We conducted a retrospective study using a multi-center cohort database of vasculitis patients. All maintenance treatment courses were divided into three groups: a RTX group, a group treated with other immunosuppressant drugs (IS) and a group receiving glucocorticoid monotherapy (GC). Read More

ANZ J Surg 2020 Jul 2. Epub 2020 Jul 2.

Anatomical Pathology Department, Waikato District Health Board, Hamilton, New Zealand.

Ann Otol Rhinol Laryngol 2020 Jul 2:3489420938827. Epub 2020 Jul 2.

Department of Otorhinolaryngology-Head and Neck Surgery, IRCCS Ospedale Policlinico San Martino, University of Genoa, Genoa, Italy.

Objective: To report presentation, diagnostic process, management and outcome of a case of autoimmune inner ear disease (AIED) related with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), treated with cochlear implantation, and review of relevant literature.

Case Presentation And Management: A retrospective case report of AIED associated with EGPA treated with cochlear implantation was described. A multi-step approach for diagnosis and confirmation of AIED and hearing rehabilitation was conducted, eventually leading to left cochlear implantation. Read More

J Obstet Gynaecol Res 2020 Jul 1. Epub 2020 Jul 1.

Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey.

Granulomatosis with polyangiitis (GPA) is a rare necrotizing autoimmune disease involving small vessel vasculitis. Pregnancies with GPA have increased rates of obstetric complications including pre-eclampsia. Differential diagnosis of GPA flares up and pre-eclampsia may be difficult and necessitates careful clinical practice. Read More

Clin Rheumatol 2020 Jul 1. Epub 2020 Jul 1.

Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, 1 Rimini Str., 124 62, Athens, Greece.

Intern Med 2020 Jun 30. Epub 2020 Jun 30.

Department of General Medicine, National Defense Medical College, Japan.

J Rheumatol 2020 Jul;47(7):1031-1032

Division of Arthritis & Rheumatic Diseases, Oregon Health & Science University, Portland, Oregon, USA.

Laryngoscope 2020 Jun 30. Epub 2020 Jun 30.

Department of Medicine, Division of Infectious Disease, Vanderbilt University Medical Center, Nashville, Tennessee, U.S.A.

Objectives/hypothesis: Characterization of the localized adaptive immune response in the airway scar of patients with idiopathic subglottic stenosis (iSGS).

Study Design: Basic Science.

Methods: Utilizing 36 patients with subglottic stenosis (25 idiopathic subglottic stenosis [iSGS], 10 iatrogenic post-intubation stenosis [iLTS], and one granulomatosis with polyangiitis [GPA]) we applied immunohistochemical and immunologic techniques coupled with RNA sequencing. Read More

World Neurosurg 2020 Jun 25. Epub 2020 Jun 25.

Department of Biomedical and Neuromotor Sciences, University of Bologna, Italy; IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.

Background: Hypertrophic pachymeningitis and spontaneous intracranial hypotension are different treatable diseases which should promptly be recognized and treated to prevent neurological sequelae. Headache and dural enhancement are the main features of both diseases, thus differentiating between these two conditions can be difficult.

Cases Description: We present two cases with headache and dural enhancement, in which the differential diagnosis was challenging at presentation because, in both cases, clear positional pain modification was not reported. Read More

Ann Rheum Dis 2020 Jun 26. Epub 2020 Jun 26.

Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of Österreichische Gesundheitskasse (ÖGK) and Research Funds of the Austrian Workers Compensation Board (AUVA),1st Medical Department, Hanusch Hospital, Wien, Austria.

Ann Rheum Dis 2020 Jun 26. Epub 2020 Jun 26.

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan.

Am J Respir Crit Care Med 2020 Jun 25. Epub 2020 Jun 25.

University of Alberta, Medicine, Edmonton, Alberta, Canada.

An international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA) is presented. ANCA, specific for myeloperoxidase (MPO), can be detected in 30-35% of EGPA patients. MPO-ANCA should be tested with antigen-specific immunoassays in any patient with eosinophilic asthma and clinical features suggesting EGPA, including constitutional symptoms, purpura, polyneuropathy, unexplained heart, gastrointestinal or kidney disease, and/or pulmonary infiltrates or hemorrhage. Read More

Tohoku J Exp Med 2020 06;251(2):117-123

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine.

Mannose-binding lectin (MBL) is a soluble pattern-recognition molecule, which plays a crucial role in the innate immune system and the activation of lectin complement pathway. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune disease affecting the small vasculatures and is characterized by the alteration of innate and adaptive immunity and complement activation. In this study, we investigated whether serum MBL is associated with disease activity of AAV, which was measured by ELISA. Read More

Ann Rheum Dis 2020 Jun 24. Epub 2020 Jun 24.

University of Cambridge, Cambridge, UK.

Objectives: Evaluation of rituximab and glucocorticoids as therapy to induce remission after relapse in ANCA-associated vasculitis (AAV) in a prospective observational cohort of patients enrolled into the induction phase of the RITAZAREM trial.

Methods: Patients relapsing with granulomatosis with polyangiitis or microscopic polyangiitis were prospectively enrolled and received remission-induction therapy with rituximab (4×375 mg/m) and a higher or lower dose glucocorticoid regimen, depending on physician choice: reducing from either 1 mg/kg/day or 0.5 mg/kg/day to 10 mg/day by 4 months. Read More

Acta Reumatol Port 2020 Jan-Mar;45(1):65-68

Hospital de Santa Maria, Centro Hospitalar Universitário de Lisboa Norte, Centro Académico de Medicina de Lisboa.

Clin Imaging 2020 Jun 18;68:71-89. Epub 2020 Jun 18.

NYU Langone Health, Department of Radiology, New York, NY, United States of America. Electronic address:

The cavernous sinus is a complex structure susceptible to a wide variety of vascular, neoplastic and inflammatory pathologies. Vascular pathologies include ICA aneurysms, carotid-cavernous fistulas, cavernous sinus thrombosis, and cavernous hemangioma. Neoplasms that involve the cavernous sinus include pituitary adenoma, meningioma, schwannoma, lymphoma, perineural tumor spread, metastases, and direct tumor invasion. Read More

J Clin Rheumatol 2020 Jun 18. Epub 2020 Jun 18.

Division of Rheumatology, Department of Internal Medicine.

J Clin Rheumatol 2020 Jun 18. Epub 2020 Jun 18.

From the Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

Rheumatol Int 2020 Aug 20;40(8):1283-1290. Epub 2020 Jun 20.

Department of Internal Medicine, School of Medicine, Pontificia Universidad Javeriana, Bogotá, Colombia.

Vasculitides associated with anti-neutrophil cytoplasmic antibodies are heterogeneous, systemic, low prevalence and high morbidity and mortality entities. They include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. In Latin America, there are few descriptive registries of these patients. Read More

Joint Bone Spine 2020 Jun 17. Epub 2020 Jun 17.

Centre de Référence des Maladies Systémiques Auto-immunes rares, Université Paris Descartes, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 27, rue du faubourg Saint Jacques, 75014, Paris, France; Institut Cochin, INSERM U1016, CNRS UMR 8104, Paris, France. Electronic address:

Granulomatosis with polyangiitis (GPA) (Wegener) is a necrotizing vasculitis combining inflammation of the vascular wall and peri- and extravascular granulomatosis. Clinically, GPA is characterized in its full form by ENT signs, lung, and kidney involvement. One of the key features of GPA is the presence of ANCAs - cytoplasmic in approximately 90% of systemic forms and in 50% of localized forms - directed against proteinase 3 in most cases. Read More

Laryngoscope 2020 Jun 20. Epub 2020 Jun 20.

Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A.

Systemic disease is an uncommon cause of subglottic stenosis (SGS). We report a case of severe SGS due to underlying eosinophilic granulomatosis with polyangiitis (EGPA) in a child presenting with isolated stridor. EGPA is a rare systemic vasculitis with very limited cases reported in the pediatric population. Read More

Heart Fail Rev 2020 Jun 20. Epub 2020 Jun 20.

Heart and Vascular Institute, West Virginian University, 1 Medical Center Dr., Morgantown, WV, 26505, USA.

Autoimmune rheumatological disorders are known to have an increased risk for cardiovascular diseases including coronary artery disease (CAD), myocarditis, pericarditis, valvulopathy, and in consequence cardiogenic shock. Data on cardiogenic shock in rheumatological diseases are scarce; however, several reports have highlighted this specific entity. We sought to review the available literature and highlight major outcomes and the management approaches in each disease. Read More

Rheumatology (Oxford) 2020 Jun 17. Epub 2020 Jun 17.

Department of Radiology, Jahrom University of Medical Science, Jahrom, Iran.

J Rheumatol 2020 Jun 15. Epub 2020 Jun 15.

National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; Department of Medicine, Weill Cornell Medicine, New York, NY; Department of Medicine, Mount Sinai Morningside and Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York, NY; Section of Cardiology, Department of Medicine, Baylor College of Medicine, Houston, TX; Department of Medicine, Mount Sinai Morningside and Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York, NY; Department of Medicine, St. Vincent's Medical Center, Bridgeport, CT; Department of Medicine, Mount Sinai Morningside and Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York, NY; Division of General Internal Medicine, Toronto General Hospital Research Institute, University of Toronto, Toronto, Ontario, Canada; Department of Healthcare Policy and Research, Weill Cornell Medicine, New York, NY; Department of Rheumatology, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY. Corresponding author: Bella Mehta, 500 East 70th Street, New York, NY, 10021. Phone: 212-606-1987. Email:

Objective: Cardiovascular diseases are serious comorbidities in patients with granulomatosis with polyangiitis (GPA). In a sample of patients hospitalized for GPA, we sought to examine trends in the burden of coronary artery disease (CAD) and its two serious manifestations, acute myocardial infraction (AMI) and heart failure (HF).

Methods: We used the National Inpatient Sample to conduct a retrospective cross-sectional analysis. Read More

Arthritis Res Ther 2020 06 15;22(1):141. Epub 2020 Jun 15.

School of Medicine, University of Occupational & Environmental Health, 1-1 Iseigaoka, Yahata-nishi, Kitakyushu, Fukuoka, 807-8555, Japan.

Objectives: B cell depletion by rituximab (RTX) is an effective treatment for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). However, peripheral B cell phenotypes and the selection criteria for RTX therapy in AAV remain unclear.

Methods: Phenotypic characterization of circulating B cells was performed by 8-color flow cytometric analysis in 54 newly diagnosed AAV patients (20 granulomatosis with polyangiitis and 34 microscopic polyangiitis). Read More

Rinsho Shinkeigaku 2020 Jun 13. Epub 2020 Jun 13.

Department of Neurology, National Hospital Organization Sendai Medical Center.

A 66-year-old woman presented with dysesthesia over the right side of her face, hypoglossal nerve dysfunction, dysphagia, and dysgeusia of the right side. A MRI scan of the brain revealed cerebral dural thickening on the right side of the skull base, and histopathological examination revealed granulomatous inflammation of the dura. Based on paranasal sinusitis, bronchodilatation, laboratory tests showing weakly positive MPO-ANCA, intact renal function, and the patient's favorable response to steroids, we diagnosed the patient with limited granulomatosis with polyangiitis (GPA). Read More

Ann Rheum Dis 2020 Jun 12. Epub 2020 Jun 12.

U.O.C. Nefrologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italia.

Gastroenterology 2020 Jun 5. Epub 2020 Jun 5.

I. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Skin Therapy Lett 2020 05;25(3):5-8

Center for Clinical Studies, Houston, TX, USA.

Small-vessel vasculitides (SVV) are a group of disorders that occur due to primarily systemic inflammation or as sequelae of an infection, malignancy, or other rheumatic disease. Arising in any organ including the skin, the clinical features of SVV encompass a variety of manifestations. A comprehensive diagnostic assessment should be performed as management protocols widely differ. Read More

J Oral Maxillofac Pathol 2020 Jan-Apr;24(1):172-175. Epub 2020 May 8.

Department of Periodontology and Implantology, Government Dental College and Hospital, Aurangabad, Maharashtra, India.

Wegener's Granulomatosis (WG) is an immunologically mediated rare multisystem disease characterized by necrotizing granulomatous inflammation affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. WG oral lesions are reported to occur in 6%-13% of patients; they were an initial presenting feature in only 2% of cases. A case of strawberry gingivitis as the first sign of WG presented with erythematous and painful localized gingival enlargement affecting the maxillary anterior region of jaw in a 17-year-old boy is reported here. Read More

J Allergy Clin Immunol Pract 2020 Jun 4. Epub 2020 Jun 4.

Rheumatology Unit, Department of Medicine DIMED, University of Padova, Padova, Italy. Electronic address:

Chest 2020 Jun 2. Epub 2020 Jun 2.

AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, F-75013, Paris, France; Centre national de références Maladies Autoimmunes systémiques rares et Centre national de références Maladies Autoinflammatoires et amylose inflammatoire; Sorbonne Universités, UPMC Université Paris 6, INSERM, UMR S 959, Immunology-Immunopathology- Immunotherapy (I3); F-75005, Paris, France. Electronic address:

Patients with autoimmune and/or inflammatory diseases (AID) are prone to serious infectious complications such as Pneumocystis Jirovecii pneumonia (PJP). In non-HIV patients, the prognosis is poorer and diagnosis tests are of lower sensitivity. Given the low incidence of PJP in AID, with the exception of granulomatosis with polyangiitis, and the non-negligible side effects of chemoprophylaxis, routine prescription of primary prophylaxis is still debated. Read More

Semin Arthritis Rheum 2020 May 15;50(4):608-615. Epub 2020 May 15.

Rheumatology, Ophthalmology and Internal medicine, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain. Electronic address:

Purpose: We assessed the efficacy and safety of biologic therapy in severe and refractory Peripheral Ulcerative Keratitis (PUK).

Design: Open-label multicenter study of biologic-treated patients with severe PUK refractory to conventional immunosuppressive drugs.

Subjects: We studied 34 patients (44 affected eyes) (24 women/10 men; mean age, 55. Read More

J Eur Acad Dermatol Venereol 2020 Jun 4. Epub 2020 Jun 4.

Departmant of Pulmonology, Iwata City Hospital, Iwata, Japan.

Cesk Patol 2020 ;56(2):68-73

The most common group of systemic vasculitides in adulthood are anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). AAV represent autoimmune systemic vasculitides and include 3 clinical phenotypes: Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), Microscopic polyangiitis (MPA) and Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). Histological features are similar to each other in all affected locations, and there are represented by necrotizing vascular inflammation of small and medium calibers, often venules, capillaries or arteriols, typically with fibrinoid vessel wall necrosis. Read More

Cesk Patol 2020 ;56(2):65-67

ANCA positive vasculitis (AAV) is a serious autoimmune disease mainly affecting small vessels in various organ systems, accompanied by the presence of ANCA antibodies in serum. AAV represents a group of the most common systemic vasculitis in adulthood, and based on clinical manifestations this disease entity includes 3 phenotypes, namely: granulomatosis with polyangiitis (formerly Wegeners granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Similar to other autoimmune diseases, AAV develops in patients with a predisposing genetic background who have been exposed to causative environmental factors, such as infections. Read More

Cureus 2020 Apr 27;12(4):e7864. Epub 2020 Apr 27.

Internal Medicine, Allegheny General Hospital, Pittsburgh, USA.

Granulomatosis with polyangiitis (GPA) formerly known as Wegener's granulomatosis, is an anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). It is an uncommon disease with an estimated prevalence of 3 in 100,000 individuals with an equal distribution in both sexes. It is characterized by necrotizing granulomatous vasculitis that primarily affects the upper and lower respiratory tracts and the kidneys. Read More

Ann Intern Med 2020 Jun 2. Epub 2020 Jun 2.

Cochin Hospital, Paris Descartes University, Paris, France (P.C., C.V., X.P., B.T., L.M., L.G.).

Background: Biannual rituximab infusions over 18 months effectively maintain remission after a "standard" remission induction regimen for patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV).

Objective: To evaluate the efficacy of prolonged rituximab therapy in preventing AAV relapses in patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) who have achieved complete remission after completing an 18-month maintenance regimen.

Design: Randomized controlled trial. Read More

Clin Case Rep 2020 May 29;8(5):862-866. Epub 2020 Feb 29.

Baton Rouge General Internal Medicine Residency Program Baton Rouge General Medical Center Baton Rouge LA USA.

Ischemic stroke is an incredibly rare manifestation of granulomatosis with polyangiitis. It is important for the clinician to be aware of this unusual complication so that efforts can be made to reduce the risk of this event. Read More

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(3):202-208. Epub 2019 May 1.

Medical University of Warsaw, Poland.

Objectives: Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is one of antineutrophil cytoplasmic autoantibody (ANCA) - associated vasculitis. In patients with GPA an increased incidence of venous thromboembolism (VTE), mainly during active disease, has been described. The aim of the present study was to assess the incidence of VTE and its relation with classic risk factors for atherosclerosis, presence of coronary artery disease (CAD), echocardiographic parameters and laboratory findings in GPA patients. Read More

Arthritis Care Res (Hoboken) 2020 May 31. Epub 2020 May 31.

F. Hoffmann-La Roche Ltd, Basel, Switzerland.

Objective: A Phase IV, open-label, prospective study was conducted to characterize the long-term safety of rituximab in a 4-year observational registry of adult patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) within the United States.

Methods: Patients initiating treatment with rituximab were evaluated every 6 months for up to 4 years. Outcomes were the incidence of serious adverse events (SAEs), infusion-related reactions (IRRs), and SAEs of specific interest, including serious infections, serious cardiac events, serious vascular events, and malignancies. Read More

Clin J Am Soc Nephrol 2020 May 29. Epub 2020 May 29.

Department of Nephrology, First Faculty of Medicine and General University Hospital, Charles University, Prague, Czech Republic.

J Med Internet Res 2020 May 14. Epub 2020 May 14.

Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia, US.

Background: The conduct of clinical trials in rare diseases utilizing the traditional centers of excellence approach remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases.

Objective: This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). Read More

Praxis (Bern 1994) 2020 ;109(7):509-512

Medizinische Klinik, Zuger Kantonsspital, Baar.

Mononeuritis Multiplex: A Diagnostic Challenge Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is a multifaceted disease. Due to the variability in vascular and organ involvement, EPGA can manifest itself very differently. We report a case of a 60-year-old patient with a known bronchial asthma, pansinusitis and newly blood eosinophilia with a rapid-onset mononeuritis multiplex. Read More

J Adv Res 2020 Jul 7;24:311-315. Epub 2020 May 7.

National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, AP-HP, Paris Descartes University, Paris, France.

Background: Skin involvement in granulomatosis with polyangiitis (GPA) is common and can appear as an initial presentation of the disease or more commonly through its course.

Case Presentation: We report a case of a 24-year-old male patient, previously diagnosed as having GPA, admitted with fever, hemoptysis, generalized hemorrhagic blisters associated with arthralgia, fatigue, myalgia, nasal crusting, and vertigo. Three weeks prior to admission, he developed erythematous papules on both elbows, and purpuric papules on both lower limbs. Read More

J Clin Rheumatol 2020 May 21. Epub 2020 May 21.

Department of Internal Medicine, Loma Linda University Medical Center, Loma Linda, CA Department of Rheumatology, Loma Linda University Medical Center, Loma Linda, CA.

J Clin Rheumatol 2020 May 22. Epub 2020 May 22.

Departments of Pathology and Laboratory Medicine.