Gastrointestinal involvement in granulomatosis with polyangiitis and microscopic polyangiitis: histological features and outcome

Latus, Joerg; Koetter, I.; Fritz, Péter; Kimmel, Martin; Biegger, Dagmar; Ott, German; Stange, Eduard F.; Amann, Kerstin; Alscher, Dominik M.; Braun, Niko

doi:10.1111/1756-185x.12203

Cited by 15 publications

(15 citation statements)

References 24 publications

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“…Although gastrointestinal involvement in patients with granulomatosis with polyangiitis (GPA) or MPA is rare, 13 and at the autopsy of this case no vasculitis was found at the infarcted bowel segment, it would not be absurd to suspect gastrointestinal involvement since in the patient’s initial symptom was abdominal pain. Moreover, there was an iron deficiency anemia, and at the autopsy a hemorrhagic infarction band was found.…”

Section: Discussionmentioning

confidence: 79%

Aortic stenosis concomitant with microscopic polyangiitis: a challenge in medical reasoning and thinking

Gutierrez¹,

Aiello²

2014

ACR

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Microscopic polyangiitis (MPA) is part of the anti-neutrophil cytoplasmic antibodies (ANCA)-related vasculitis, which usually presents as renal pulmonary syndrome. It is defined as a pauci-immune necrotizing small vessel vasculitis, which usually affects the kidneys, followed by the lungs. It also presents systemic symptoms. The etiology of MPA is still unclear, but evidence reinforces the autoimmune mechanisms as the main etiopathogenic factor. Aortic valve stenosis (AS) is not an uncommon disease whose etiology varies according to geographical differences and the patient’s age. The natural history of AS begins with a prolonged asymptomatic period, but when symptomatic, respiratory failure is one of its main clinical presentations. The authors present the case of a 55-year-old woman who was admitted with the diagnosis of renal failure, anemia, and a cardiac murmur. The patient had been recently diagnosed with pneumonia. During hospitalization, diagnostic workup disclosed a normal kidney size as well as parenchymal thickness. A renal biopsy was undertaken but the specimen was exiguous, showing 4 sclerotic glomeruli and 1 glomerulus with crescentic glomerulonephritis. The search for ANCA was positive. The investigation of the cardiac murmur disclosed AS. The patient, on hemodialysis, presented episodes of respiratory failure, which was interpreted as acute pulmonary edema, but a suspicion of ANCA-related pulmonary renal syndrome was raised. However, the aortic valve replacement was prioritized. While awaiting cardiac surgery, the patient died because of respiratory insufficiency. Autopsy findings concluded that MPA with pulmonary hemorrhage due to vasculitis was the immediate cause of death. Although AS was present at autopsy and classified as moderate/severe, this lesion was a bystander in the process of this patient’s end of life, demonstrating the value of autopsy for medical learning and reasoning purposes.

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Section: Discussionmentioning

confidence: 79%

Aortic stenosis concomitant with microscopic polyangiitis: a challenge in medical reasoning and thinking

Gutierrez¹,

Aiello²

2014

ACR

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“…6 – 8 Intestinal involvement has comparatively been described more frequently, often with mesenteric ischemia, ulceration, bleeding, or abdominal perforation. 3 , 9 , 10…”

Section: Discussionmentioning

confidence: 99%

“…Patients have higher Birmingham Vascular Assessment Scores and increased need for surgery with GI GPA, often because of acute abdomen or intractable bleeding. 9 , 10 One review found 54.5% mortality with GI involvement, and all cases reviewed required surgery. 13 Furthermore, GI GPA has been associated with higher mortality.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal Hemorrhage With Gastritis and Pancolitis as the Sole Presentation for Granulomatosis With Polyangiitis Flare

et al. 2022

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Granulomatosis with polyangiitis (GPA), previously known as Wegener granulomatosis, is a rare small vessel vasculitis affecting mainly Whites. The prevalence of GPA in the United States is estimated to be 3 of 100,000 individuals. Classically, GPA affects upper airways, lungs, and kidneys, with the upper airways being the most common site. Occasionally, other organs affected by GPA include eyes, skin, joints, and the nervous system. The gastrointestinal system is rarely affected; however, some cases have been reported. In this case report, we present a patient with hemorrhagic gastritis and pancolitis consistent with GPA and discuss features from the literature of gastrointestinal manifestations in patients with GPA.

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“… 12 – 18 Gastrointestinal symptoms were mainly acute abdominal pain, but other symptoms including nausea or vomiting, diarrhea, and gastrointestinal bleeding can be presenting symptoms. 14 , 19 – 21 In addition, gastric outlet obstruction, intussusception, and duodenal necrosis have been reported. 22 , 23 In our study, the most common primary vasculitis with UGI involvement was HSP (56.8%) and 76.4% of patients with primary vasculitis had gastrointestinal symptoms, abdominal pain being the most common.…”

Section: Discussionmentioning

confidence: 99%

“…Gastrointestinal involvement of systemic vasculitis has been reported to be associated with severe complications and poor outcomes especially in patients with polyarteritis nodosa, microscopic angiitis, and granulomatosis with polyangiitis. 14 , 25 – 28 Therefore, accurate diagnosis and early treatment is important to avoid fatal outcomes, and endoscopic examination helps to define the site and extent of involvement and to assess prognosis. However, the diagnosis of vasculitis is difficult and endoscopic findings do not always correlate with presenting symptoms.…”

Section: Discussionmentioning

confidence: 99%

Endoscopic Findings of Upper Gastrointestinal Involvement in Primary Vasculitis

et al. 2016

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Background/AimsGastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis.MethodsClinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records.ResultsOf 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement. Of these, 21 cases (14.2%) were classified as large-vessel vasculitis, 17 cases (11.5%) as medium-vessel vasculitis, and 110 cases (74.3%) as small-vessel vasculitis. According to the specific diagnosis, IgA vasculitis (Henoch-Schönlein purpura) was the most common diagnosis (56.8%), followed by Takayasu arteritis (14.1%), microscopic polyangiitis (10.1%), and polyarteritis nodosa (6.8%). Gastrointestinal symptoms were present in 113 subjects (76.4%), with abdominal pain (78.8%) the most common symptom. Erosion and ulcers were striking endoscopic features, and the second portion of the duodenum was the most frequently involved site. Biopsy specimens were obtained from 124 patients, and only eight (5.4%) presented histopathological signs of vasculitis.ConclusionsDiagnosis of vasculitis involving the upper gastrointestinal tract is difficult. Because of the widespread use of endoscopy, combining clinical features with endoscopic findings may facilitate making appropriate diagnoses; however, the diagnostic yield of endoscopic biopsy is low.

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Gastrointestinal involvement in granulomatosis with polyangiitis and microscopic polyangiitis: histological features and outcome

Abstract: GI involvement in GPA and MPA is rare. Therapy directed at T cells might be an alternative treatment option.

Cited by 15 publications

References 24 publications

Aortic stenosis concomitant with microscopic polyangiitis: a challenge in medical reasoning and thinking

Aortic stenosis concomitant with microscopic polyangiitis: a challenge in medical reasoning and thinking

Gastrointestinal Hemorrhage With Gastritis and Pancolitis as the Sole Presentation for Granulomatosis With Polyangiitis Flare

Endoscopic Findings of Upper Gastrointestinal Involvement in Primary Vasculitis

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