Granulomatosis with Polyangiitis with Intestinal Perforations as Complications: Case Report
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2019, Vol 12, Issue 5
Abstract
Granulomatosis with Polyangiitis (GPA) is a systemic vasculitis which clinical manifestations are diverse, usually it shows involvement in pulmonary and nephrological level. The clinical case of a 36 years old male with the mentioned condition and manifestation of gastrointestinal involvement is presented.Granulomatosis with polyangiitis (previously known as Wegener’s Granulomatosis) is a necrotizing vasculitis of small blood vessels with multiple system impact characterized by necrosis and granulomatous inflammation, it was described for the first time by Klinger in 1931 and later by Wegener in 1936 [1]. It is a systemic clinical-pathological entity mediated by immune mechanisms. The most common clinical manifestations are of otorhinolaryngological, respiratory and renal origin. The gastrointestinal involvement is rare and a poor prognosis marker [2,3]. The clinical case of a patient with granulomatosis with polyangiitis, intestinal perforation and secondary peritonitis is presented in Figure 1.A 36 years old male without serious pathological elements in his past history presents a clinical picture of one-month evolution with arthralgia, fever, dyspnea on medium exertion associated with hemoptysis and weight loss of 7 kilograms. He goes into hospitalization, in his assessment, purpuric injuries are found on the skin, renal failure, anemia, leukocytosis and positive tests for c-ANCA antibodies are documented. In the computed tomography of the chest hyper dense paramilitary objects and ground glass areas are observed. The bronchoscopy shows diffuse alveolar bleeding and inflammatory changes. Kidney biopsy with proliferative extra capillary glomerulonephritis with fibrinoid necrosis. He is given 1 gram of methylprednisolone per day in 3 doses and in the 4th day, 1 gram of cyclophosphamide is added. 24 hours later he develops a septic shock. A laparotomy is performed, and colonic perforation is found, showing torpid post operatory evolution with eventual death. Final histopathological diagnosis: Intestinal perforation with transluminal enteritis and acute peritonitis.The patient in the case was diagnosed with granulomatosis with polyangiitis based on clinical criteria, positive tests for c-ANCA and kidney biopsy consistent with vasculitis of small blood vessels. The literature documents gastrointestinal involvement is rare, nevertheless it is the cause of death IN 12% of the cases of GPA, in recent reports the gastrointestinal illness includes: submucosal edema, ulcers, hemorrhage, mesenteric ischemia, intestinal obstruction and perforation [4-6]. Colonic perforation associated with cytomegalovirus infection in immunocompromised patients (kidney transplant, systemic erythematosus lupus, HIV infection) precipitated by treatment with steroids has been documented in literature, however literature regarding GPA has not been found [7]. In patients with perforation on the left side of the colon, it may be difficult to distinguish between diverticulitis precipitated by vasculitis o as a complication of the immunosuppressive therapy, or a combination of both.
Authors and Affiliations
Cobos Quevedo Orestes de Jesús, Rentería Guevara Cynthia Mireya, Ramírez Montelongo Sandra María
Keywords
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- EP ID EP587475
- DOI 10.26717/BJSTR.2019.12.002309
- Views 171
- Downloads 0
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