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Intestinal perforation as a first presentation of granulomatosis with polyangiitis: unusual case report

Introduction: Granulomatosis with polyangiitis (GPA) vasculitis typically involves upper and lower airways and kidneys. Gastrointestinal involvement is rare, clinically reported as esophageal involvement, gastrointestinal hemorrhage, intestinal perforation, colitis, and pancreatitis. Case presentati...

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Detalles Bibliográficos
Autores principales: Khalayli, Naram, Aldeeb, Maria, Abouharb, Dani, Abouharb, Lana, Kudsi, Maysoun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10686004/
https://www.ncbi.nlm.nih.gov/pubmed/38033412
http://dx.doi.org/10.1093/omcr/omad127
Descripción
Sumario:Introduction: Granulomatosis with polyangiitis (GPA) vasculitis typically involves upper and lower airways and kidneys. Gastrointestinal involvement is rare, clinically reported as esophageal involvement, gastrointestinal hemorrhage, intestinal perforation, colitis, and pancreatitis. Case presentation: We present a 36 old man, with intestinal perforation, laterally diagnosed as granulomatosis with polyangiitis. Discussion: Only a few cases of intestinal perforation have been reported in the medical literature. GI symptoms may be present after the disease diagnosis in years. Intestinal perforation usually required surgery. The frequent kidney involvement of GPA is rapidly progressive glomerulonephritis, presented as acute kidney injury, usually accompanied by GI symptoms. Cyclophosphamide plus corticosteroids remain the effective therapy. The patient with GPA had a normal life expectancy due to the advances in treatment. Renal involvement and GI manifestations are considered bad prognosis predictors. Conclusion: This case report illustrates the need to consider intestinal perforation in patients with granulomatosis with polyangiitis, early surgical intervention and appropriate immunosuppressive therapy can be lifesaving.