Targeted Immunotherapy Strategies in ANCA-Associated Vasculitis
This article reviews the current role for targeted immunotherapy in AAV, its validated indications, and avenues for further development. Rituximab is a validated induction treatment for GPA and severe MPA. Rituximab in these indications is not less effective than cyclophosphamide and is particularly useful in patients with refractory or relapsing disease, women of childbearing potential, and patients previously treated with cyclophosphamide. Rituximab is more effective than cyclophosphamide for treating relapses. For remission maintenance therapy, which is indispensable, rituximab has been proven superior over conventional...
Source: Joint Bone Spine - September 8, 2018 Category: Orthopaedics Source Type: research

Clinical manifestations of granulomatosis with polyangiitis: key considerations and major features.
CONCLUSION: Major advances in the understanding and treatment of GPA over recent decades have contributed to the notable decline in morbidity and mortality of patients. The ultimate goal is an improved prognosis through outcome measures which assesses the disease control with minimal adverse effects of intensive immunosuppressive regimens, an integral part of the clinical approach to improve the quality of life of GPA patients. PMID: 30071173 [PubMed - as supplied by publisher] (Source: Postgraduate Medicine)
Source: Postgraduate Medicine - August 4, 2018 Category: Internal Medicine Tags: Postgrad Med Source Type: research

Granulomatosis with polyangiitis: clinical course and outcome of 60 patients from a single center in South India
AbstractGranulomatosis with polyangiitis (GPA) previously known as Wegener ’s granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Patients who fulfilled any two of the four criteria proposed by the American College of Rheumatology, and those with clinical features of GPA with ANCA positivity and histopathological confirmation, were included in the study. Disease activity and damage were asse...
Source: Clinical and Experimental Medicine - August 1, 2018 Category: Research Source Type: research

A case of rheumatoid nodules in the buccal subcutaneous : A novel case report of review and the literature
Publication date: May 2018Source: Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology, Volume 30, Issue 3Author(s): Kazuhiro Ogi, Akihiro Miyazaki, Shota Shimizu, Masato Abe, Ryoichi Genba, Sho Miyamoto, Koyo Nishiyama, Hiroyoshi HiratsukaAbstractMethotrexate (MTX) has been reported to be effective for the treatment of rheumatoid arthritis (RA) when a weekly low dose is administered to patients. We carried out microscopic examination of a biopsied specimen suspected of being Wegener’s granulomatosis, IgG4-related disease, or malignant lymphoma. Finally we diagnosed a rheumatoid nodule because his medical h...
Source: Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology - July 10, 2018 Category: ENT & OMF Source Type: research

Thoracic manifestation of Wegener's granulomatosis: Computed tomography findings and analysis of misdiagnosis.
Authors: Li J, Li C, Li J Abstract The aim of the present study was to investigate the computed tomography (CT) manifestations of Wegener granulomatosis (WG) in the chest and potential reasons for misdiagnosis. Conventional CT scans and clinical data of 45 patients with WG were retrospectively analyzed. Patients typically presented with multiple system involvement, primarily in the upper and lower respiratory tract. The incidence of thoracic involvement was 75.56% (34/45). Radiographic features were varied between cases in the present study, with the most common features being numerous cavitary nodules and...
Source: Experimental and Therapeutic Medicine - June 15, 2018 Category: General Medicine Tags: Exp Ther Med Source Type: research

Severe localised granulomatosis with polyangiitis (Wegener ’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review
Granulomatosis with polyangiitis (GPA, formerly Wegener ’s granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels. Cranial involvement can result in cranial nerve palsies and, rarel... (Source: BMC Neurology)
Source: BMC Neurology - May 1, 2018 Category: Neurology Authors: James E. Peters, Vivek Gupta, Ibtisam T. Saeed, Curtis Offiah and Ali S. M. Jawad Tags: Case report Source Type: research

An ugly nose and a Nazi father: Bad premise for a cardiac rheumatic disorder
The rheumatic disorder reported on by Abe and coworkers1 in this issue of the Journal has long been labeled as Wegner granulomatosis, ever since Friedrich Wegener,2 a Nazi pathologist working in Poland,3 reported 3 cases in 1937, describing the pathologic disorders as vasculitis. Actually, this disease had first been reported by Wegner's friend, a Berlin pathologist named Heinz Klinger,4 who in 1932 described a case of destructive sinusitis, lung abscesses, and uremia, characterized by angiitis and granulomas, in a report entitled, “Borderline Forms of Periarteritis Nodosa.” Today, this rheumatic disorder is known as g...
Source: The Journal of Thoracic and Cardiovascular Surgery - April 13, 2018 Category: Cardiovascular & Thoracic Surgery Authors: Massimiliano Foschi, Fabrizio Costantino Tancredi, Michele Di Mauro Tags: Editorial commentary Source Type: research

A case of rheumatoid nodules in the buccal subcutaneous : A novel case report of review and the literature
Publication date: Available online 28 March 2018 Source:Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology Author(s): Kazuhiro Ogi, Akihiro Miyazaki, Shota Shimizu, Masato Abe, Ryoichi Genba, Sho Miyamoto, Koyo Nishiyama, Hiroyoshi Hiratsuka Methotrexate (MTX) has been reported to be effective for the treatment of rheumatoid arthritis (RA) when a weekly low dose is administered to patients. We carried out microscopic examination of a biopsied specimen suspected of being Wegener’s granulomatosis, IgG4-related disease, or malignant lymphoma. Finally we diagnosed a rheumatoid nodule because his med...
Source: Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology - March 29, 2018 Category: ENT & OMF Source Type: research

Wegener ’s granulomatosis of the heart
(Source: The Journal of Thoracic and Cardiovascular Surgery)
Source: The Journal of Thoracic and Cardiovascular Surgery - March 10, 2018 Category: Cardiovascular & Thoracic Surgery Authors: Noriyuki Abe, Hiroaki Takahashi, Takeshi Inoue, Hiroshi Tanaka, Yutaka Okita Source Type: research

Wegener granulomatosis of the heart
We report here 2 cases of GPA in which a central fibrous body was involved. The institutional ethics committee provided appropriate approval. (Source: The Journal of Thoracic and Cardiovascular Surgery)
Source: The Journal of Thoracic and Cardiovascular Surgery - March 10, 2018 Category: Cardiovascular & Thoracic Surgery Authors: Noriyuki Abe, Hiroaki Takahashi, Takeshi Inoue, Hiroshi Tanaka, Yutaka Okita Tags: Adult: Aortic Valve: Case Report Source Type: research

Granulomatosis with polyangiitis: clinical course and outcome of 60 patients from a single center in South India
AbstractGranulomatosis with polyangiitis (GPA) previously known as Wegener ’s granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Patients who fulfilled any two of the four criteria proposed by the American College of Rheumatology, and those with clinical features of GPA with ANCA positivity and histopathological confirmation, were included in the study. Disease activity and damage were asse...
Source: Clinical and Experimental Medicine - February 28, 2018 Category: Research Source Type: research

Granulomatosis with polyangiitis presenting as facial nerve palsy in a teenager
Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e.g. (Source: International Journal of Pediatric Otorhinolaryngology)
Source: International Journal of Pediatric Otorhinolaryngology - February 7, 2018 Category: ENT & OMF Authors: James C. Wang, Brittany A. Leader, Ryan A. Crane, Bernadette L. Koch, Matthew M. Smith, Stacey L. Ishman Tags: Case Report Source Type: research

Serum 25-hydroxyvitamin D levels in patients with Granulomatosis with Polyangiitis: association with respiratory infection
CONCLUSIONS: Lower 25OHD levels were associated with respiratory infection but not disease activity in granulomatosis with polyangiitis patients. Our data suggest that hypovitaminosis D could be an important risk factor for respiratory infection in granuloma tosis with polyangiitis patients. (Source: Clinics)
Source: Clinics - January 9, 2018 Category: General Medicine Source Type: research