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Granulomatosis with Polyangiitis

Overview
Symptoms
Diagnosis
Treatments
Causes
Prognosis
Research
Personal Stories

Overview

Wegeners Granulomatosis KaleidoscopeGranulomatosis 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.

Symptoms

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.

Diagnosis

Significance of PR3–ANCA positivity in eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss). This suggests that PR3–ANCA EGPA could be a particular form of PR3–ANCA–associated vasculitis. PubMed, Rheumatology (Oxford), 2020 Dec 21;keaa805.

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.

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.

Treatments

Long-Term Safety of Rituximab in Granulomatosis With Polyangiitis (GPA) or Microscopic Polyangiitis (MPA). These findings provide clinicians with long-term, practice-level safety data for rituximab in the treatment of GPA or MPA. PubMed, Arthritis Care Res (Hoboken), 05/31/2020.

(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)

Causes

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.

Prognosis

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.

Research

Case Report: Severe biventricular thrombosis in eosinophilic granulomatosis with polyangiitis (EGPA). Cardiac involvement in EGPA, a rare cause of heart failure, can manifest as severe biventricular thrombosis and severe tricuspid regurgitation, resulting in heart failure with preserved ejection fraction. PubMed, Eur Heart J Case Rep. (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.

Personal Stories

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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