Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
Journal
Revista da Associacao Medica Brasileira (1992)
ISSN: 1806-9282
Titre abrégé: Rev Assoc Med Bras (1992)
Pays: Brazil
ID NLM: 9308586
Informations de publication
Date de publication:
Jul 2020
Jul 2020
Historique:
received:
19
12
2019
accepted:
08
12
2019
entrez:
27
8
2020
pubmed:
28
8
2020
medline:
10
9
2020
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 32844923
pii: S0104-42302020000700904
doi: 10.1590/1806-9282.66.7.904
pii:
doi:
Substances chimiques
Methylprednisolone
X4W7ZR7023
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM