ANCA-associated vasculitis

Multiple Organ ◽

Histological Lesion ◽

Considerable Heterogeneity ◽

Organ Systems ◽

Strauss Syndrome ◽

Acute And Chronic Inflammation ◽

The ANCA-associated vasculitides (AAV) are a grouping of three syndromes of acute and chronic inflammation characterized by their clinical and histological phenotypes, which are associated with circulating antineutrophil cytoplasm autoantibodies (ANCA). They comprise: granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis); microscopic polyangiitis (MPA); and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). The defining histological lesion is a microscopic vasculitis affecting arterioles, capillaries, or venules associated with few or no deposits of immunoglobulin or complement. Granulomata, involving or close by blood vessels, are commonly present in GPA. These diseases involve multiple organ systems with considerable heterogeneity in extent and severity of organ involvement between patients, and overlapping clinical and histological features between syndromes.

Childhood-onset Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss Syndrome): A Contemporary Single-center Cohort

The Journal of Rheumatology ◽

10.3899/jrheum.120808 ◽

2013 ◽

Vol 40 (6) ◽

pp. 929-935 ◽

Cited By ~ 38

Author(s):

Samantha Gendelman ◽

Andrew Zeft ◽

Steven J. Spalding

Keyword(s):

Upper Airway ◽

Initial Therapy ◽

Childhood Onset ◽

Churg Strauss Syndrome ◽

Organ Systems ◽

Strauss Syndrome ◽

Objective.To date only 38 cases of childhood-onset eosinophilic granulomatosis with polyangiitis (cEGPA; formerly Churg-Strauss syndrome) have been reported. Additional patients with cEGPA could enhance the understanding of this rare and life-threatening condition. Our objectives were (1) to determine the frequency of specific organ system involvement; (2) to examine initial therapeutic regimen; and (3) to document disease and therapy-related morbidity in a contemporary cohort of patients with cEGPA.Methods.Retrospective review of patients evaluated at the Cleveland Clinic between 2003 and 2011 who met either American College of Rheumatology or Lanham criteria for EGPA and whose age was < 18 years at symptom onset.Results.Nine patients (8 female; 7 white) were identified. Median age at onset of rhinitis/asthma symptom was 13 years and median age at diagnosis of cEGPA was 15 years. All patients demonstrated eosinophilia, upper airway disease (allergic rhinitis, chronic sinusitis, and/or nasal polyps), and pulmonary involvement. Other frequently involved organ systems included musculoskeletal (67%), gastrointestinal (67%), cutaneous (67%), neurologic (56%), and cardiac (44%). Antineutrophil cytoplasmic antibody (ANCA) serologies were negative in all patients. The medications used most frequently for initial therapy included oral (44%) or intravenous corticosteroids (56%) and azathioprine (67%). Disease or therapeutic complications occurred in half of the cohort and included heart failure, stroke, and sequela from longterm, high-dose steroids.Conclusion.Eosinophilia, in combination with upper airway, pulmonary, musculoskeletal, neurologic, and cardiac manifestations, is frequently observed in cEGPA. ANCA titers are often negative. Steroids are the mainstay of initial therapy but steroid-related side effects occur regularly.

Omalizumab-associated eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Annals of Allergy Asthma & Immunology ◽

10.1016/j.anai.2016.12.003 ◽

2017 ◽

Vol 118 (3) ◽

pp. 372-374.e1 ◽

Cited By ~ 5

Author(s):

Salik Nazir ◽

Niranjan Tachamo ◽

Shoaib Bilal Fareedy ◽

Muhammad Sohail Khan ◽

Saroj Lohani

Keyword(s):

Churg Strauss Syndrome ◽

Strauss Syndrome ◽

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Revista da Associação Médica Brasileira ◽

10.1590/1806-9282.66.7.904 ◽

2020 ◽

Vol 66 (7) ◽

pp. 904-907

Author(s):

Gabriela Venade ◽

Cátia Figueiredo ◽

Catarina Almeida ◽

Nídia Oliveira ◽

Luis Costa Matos

Keyword(s):

Complex Disease ◽

Previous History ◽

Specific Treatment ◽

Disease Diagnosis ◽

Churg Strauss Syndrome ◽

Strauss Syndrome ◽

SUMMARY Churg–Strauss syndrome, Eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic vasculitis that affects small– to medium-sized vessels. It is rare and part of the Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) group. We present a 37-year-old man, with a previous history of asthma, that was sent to the ED due to 2 weeks of productive cough, occasional dyspnea on exertion, fever (one week), asthenia, and anorexia. Upon physical examination, he was subfebrile and tachycardic. He had leukocytosis (17.00 x10^9/L) and eosinophilia of 20.0 % (3.4 X10^9/L), creatinine level of 1.5 mg/dL, subtle elevation on liver function tests and CRP of 10.82mg/dL. On Chest X-Ray, there was infiltrate on the right pulmonary base. Due to a strong suspicion of EGPA, he was started on 80mg of prednisolone from admission. ANCA MPO was positive, with the remaining auto-immune study negative. He underwent Thorax CT (under corticotherapy) without relevant changes, as well as bronchoalveolar lavage, without macroscopic signs of alveolar hemorrhage. Because of active urinary sediment, nephrotic proteinuria (6.5g/24h), and acute renal failure he underwent a renal biopsy, which revealed pauci-immune crescentic glomerulonephritis, with predominantly acute findings (in the context of ANCA-MPO Vasculitis – EGPA). After the biopsy, he received three 1g methylprednisolone pulses and was started on Cyclophosphamide. He remained asymptomatic and renal function was restored. This case highlights the importance of integrating all findings in one clinical scenario to prevent a more complex disease diagnosis, with a specific treatment, from being missed.