Granulomatosis with polyangiitis (GPA) (formerly Wegener's Granulomatosis) is a type of vasculitis (inflammation of the blood vessels) caused by an abnormal amount of the antineutrophil cytoplasmic antibodies (ANCAs) circulating in the blood. The ANCAs cause an autoimmune attack on the small and medium-size blood vessels. This can damage vital organs and become life threatening. Like many autoimmune diseases, GPA may require immunosuppression. (Also see Vasculitis and ANCAs)
Granulomatosis with Polyangiitis (Wegener's): An Alternative Name for Wegener's Granulomatosis. This article announces the newly proposed name, outlines the reasons for seeking a new disease name, and explains the rationale for the proposed name. Journal of the American Society of Nephrology (JASN).
Increased Risk of Autoimmune Disease in Families with Granulomatosis with polyangiitis (GPA) (formerly Wegener's Granulomatosis) Relatives of patients with GPA are at increased risk of being diagnosed with other autoimmune/inflammatory diseases, indicating shared susceptibility between GPA and other autoimmune/ inflammatory disease. PubMed, J Rheumatol.
Systemic autoimmune diseases form a diverse group which includes: systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), scleroderma, dermato-polymyositis, granulomatosis with polyangiitis (GPA), Sjogren's Syndrome. The frequency and extent of cardiac pathology positively correlated with the detection of antiphospholipid antibodies. No such relationship was observed in patients with the presence of very high titers of antinuclear antibodies (anti-dsDNA). PubMed, Pol Arch Med Wewn.
Granulomatosis with polyangiitis (formerly Wegener's Granulomatosis). This is an uncommon disorder that causes inflammation of your blood vessels, which in turn restricts blood flow to various organs. Most commonly granulomatosis with polyangiitis affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. Mayo Clinic.
Ear, nose and throat involvement in granulomatosis with polyangiitis (GPA): how it presents and how it determines disease severity and long–term outcomes. Our data confirms that ENT involvement is not only one of the key clinical features of GPA, but also could point out a milder GPA subset with lower renal involvement and lower mortality rate, irrespectively to ANCA status. PubMed, Clin Rheumatol 02/20/2018.
The usefulness of histopathological examinations of non–renal biopsies in the diagnosis of granulomatosis with polyangiitis. Histopathological examination of the affected tissues remains the gold standard of the diagnosis of GPA. PubMed, Reumatologia. 2017;55(5):230-236.
Value of non–identified ANCA (non–PR3, non–MPO) in the diagnosis of granulomatosis with polyangiitis (GPA) (formerly Wegener's granulomatosis). GPA with non–MPO, non–PR3 ANCAs is relatively rare and our study suggests that GPA with nonidentified ANCA differs from GPA with identified ANCA by the frequency of localized forms. PubMed, Acta Clin Belg, 2017 Jan 9:1-5.
Case Report: Gingival hyperplasia as first sign of recurrence of granulomatosis with polyangiitis (GPA) (formerly Wegener's granulomatosis). The often overlooked oral manifestation may be interpreted as the first evidence of resurgent GPA in general and therefore could be pathognostic for the disease. PubMed, BMC Oral Health.
(Case Report) Rituximab (RTX) for eosinophilic granulomatosis with polyangiitis with severe vasculitic neuropathy: review of current clinical evidence. RTX seems to be effective in cases of severe EGPA refractory to standard of care immunosuppressive treatment, although support comes from case reports and non-controlled studies. PubMed, Semin Arthritis Rheum. (Also see Biologic Agents)
Case Report: Headache Linked to Intracranial Hypertension and Hypertrophic Pachymeningitis (HP) as the Initial and Dominant Presentation of Granulomatosis With Polyangiitis (GPA). Recent studies are reporting an increased frequency of HP and are distinguishing a granulomatous and a vasculitic phenotype, with different localization and relapse rates, that may eventually constitute a different clinical spectrum of GPA. PubMed, Headache.
Red Blood Cell Distribution Width Can Predict Vasculitis Activity and Poor Prognosis in Granulomatosis with Polyangiitis (GPA). RDW predicts vasculitis activity in GPA, and ≥RDW 15.4% at diagnosis may increase the risk of severe GPA at diagnosis and predict refractory diseases during follow-up. PubMed, Yonsei Med J, 2018 Mar;59(2):294-302.
Central nervous system (CNS) involvement of granulomatosis with polyangiitis (GPA): a single–center retrospective study. Based on our results, CNS involvement did not affect the long–term outcomes of GPA patients. PubMed, Clin Rheumatol, 2018 Mar;37(3):737-747.
Cardiac Involvement in Granulomatosis with Polyangiitis (GPA) : a magnetic resonance imaging study of 31 consecutive patients. This unique non–invasive technique may provide information with important clinical implications for the accurate early assessment of cardiac lesions in GPA patients and for detecting cumulative, irreversible damage. PubMed, Rheumatology (Oxford), 2017 Jun 1;56(6):947-956 (Also see Cardiac (Heart) Involvement)
In situ evidence of pulmonary endothelial activation in patients with granulomatosis with polyangiitis (GPA) and systemic sclerosis (SSc). These observations are an evidence of in situ pulmonary vascular endothelial activation in lesions of GPA and SScl, adding information to the pathogenic knowledge of both diseases. PubMed, Lung.
Luca: Difficult Diagnosis, Incomplete (Italy) These are my symptoms: joint pain, bone pain, fever, the inside of my mouth is irritated, as if I had burned it…
(Italiano) Luca: Dificulta Diagnosa Incompleta (Italia) Ma io stó davvero male e non so piú che fare, vivo davvero male. Addirittura mi hanno detto che sono sintomi immaginari, ma non é cosí…
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