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Wegener's Granulomatosis

Author: Jo Frowde. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Symptoms
Diagnosis
Treatments
Causes
Prognosis
Research
Personal Stories

Overview

Wegeners Granulomatosis KaleidoscopeWegener's Granulomatosis (WG) 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, WG may require immunosuppression. (Also see Vasculitis and ANCAs)

Increased Risk of Autoimmune Disease in Families with Wegener's Granulomatosis (WG). Relatives of patients with WG are at increased risk of being diagnosed with other autoimmune/inflammatory diseases, indicating shared susceptibility between WG 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, Wegener's granulomatosis, 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 Wegener's granulomatosis affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. Mayo Clinic.

Diagnosis

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) (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) (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

Successful use of Rituximab in a patient with recalcitrant multisystemic eosinophilic granulomatosis with polyangiitis (EGPA). Few cases are reported in the literature about the role of Rituximab in EGPA, of which only one described retinal artery occlusion as a presentation of a recently treated patient with EGPA. PubMed, BMJ Case Rep, 05/15/2015. (Also see Biologic Agents)

(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): clinical-radiological presentation distinguishes different outcomes. The clinical-radiological phenotype distinguished different long-term outcomes in patients with GPA and CNS involvement. PubMed, Rheumatology (Oxford), 2015 Mar;54(3):424-32.

Research

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, 2015 Jun;193(3):355-9.

Cardiac Involvement in Granulomatosis with Polyangiitis (GPA). Cardiac involvement in GPA is rare and heterogeneous and was not associated with a higher rate of relapse or premature death. PubMed, J Rheumatol, 05/01/2015. (Also see Cardiac (Heart) Involvement)

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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