Granulomatosis with Polyangiitis Complicated with Gastrointestinal Perforation: A Case Report and Review of Literature

Abstract

summaryA 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes. Gastrointestinal perforation in GPA is a rare complication, and we examined the clinical features, treatment contents, and prognosis of GPA with gastrointestinal perforation from this case and previous reports. Lung involvements were complicated in all reported cases. Gastrointestinal perforations in GPA were frequent in the small intestine, occurred just before and immediately after the start of treatment, and were severe involvement with poor prognosis because of the high mortality rate (46.7%). The frequency of ear, nose and upper respiratory tract lesions in the surviving group was significantly higher than in the dead group (survival 87.5%, death 28.3%, P = 0.041). IVCY were more frequently used in the surviving group (62.5%) than the death group (16.7%), but it was not significantly. GPA complicated with gastrointestinal perforation is a severe condition with poor prognosis, but there is a possibility to improve prognosis by early diagnosis and early initiation of strong treatment.

Ileal perforation involvement in Wegener granulomatosis comorbid with COVID-19 infection: A case report and review of the literature

“…One of their patients suffered an ileal perforation with diffuse peritonitis and, consequently, underwent emergency surgery to remove part of the ileum and then achieved remission following an early postoperative plasma exchange together with a combination of intravenous cyclophosphamide and glucocorticoids followed up with azathioprine maintenance therapy. Therefore, as Kiboshi et al [2] emphasized that clinicians should be aware of different presentations of GPA with intestinal perforation to improve prognoses by securing an early diagnosis and appropriate intensive treatment.…”

“…The clinical characteristics of those 25 patients and of the patient in the present case are summarized in Table 2. [2,13–35] These patients had a wide-ranging age-of-onset (19–69 years) with a predominance of young adult patients and a male-to-female ratio of 18:8. This sample of 26 patients with GPA includes 9 patients who were PR3-ANCA–positive, 10 patients who were c-ANCA–positive, 2 patients who were positive for both PR3-ANCA and c-ANCA and a single patient who had the anti-myeloperoxidase antibody.…”

Manifestations of the gastrointestinal tract in systemic rheumatic diseases: A narrative review

“…Помимо трофических нарушений, негативное влияние на ЖКТ при этих заболеваниях оказывают гранулематозные изменения (ГПА) и эозинофильная инфильтрация подслизистого слоя (ЭГПА). В ряде случаев данная патология приводит к развитию эрозивно-язвенных изменений, кровотечениям и перфорации [34][35][36][37][38].…”

Granulomatosis with Polyangiitis with Intestinal Perforations as Complications: Case Report

“…The use of immunosuppressants, mainly steroids in high doses has been associated as the etiology of intestinal necrosis and the perforation, this hypothesis is backed up by the absence of vasculitis in the histopathological report of our case even though the treatment could also alter the histopathological results. On the other hand, spontaneous perforation even without treatment has been observed and also healing of the intestinal perforation after the administration of immunosuppressants has been documented, besides, a successful treatment with rituximab in patients that presented intestinal perforation as complication has been observed [3,[7][8][9].…”

“…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.…”