Microscopic polyangiitis complicated with ileal involvement detected by double-balloon endoscopy: a case report
© Fukushima et al; licensee BioMed Central Ltd. 2013
Received: 11 July 2012
Accepted: 28 February 2013
Published: 2 March 2013
Microscopic polyangiitis is characterized by pauci-immune, necrotizing small-vessel vasculitis and an anti-neutrophil cytoplasmic antibody-associated vasculitis. Although gastrointestinal involvement in microscopic polyangiitis is not rare, endoscopic observation of it is extremely rare. To the best of our knowledge, this is the first case report of small intestinal involvement in microscopic polyangiitis detected and followed up by double-balloon endoscopy.
A 70-year-old Japanese woman was transferred to our hospital for close examination of suspected small intestinal lymphoma. Retrograde double-balloon endoscopy revealed various forms of ulcers with redness and edema in the ileum. Histological findings suggested ischemic changes. Because mononeuritis multiplex and a fever spike appeared later, vasculitis was suspected. The perinuclear anti-neutrophil cytoplasmic antibody titer was elevated. Nerve biopsy results suggested vasculitis. From these findings, microscopic polyangiitis was diagnosed. It was suggested that microscopic polyangiitis caused the intestinal involvement. Intravenous pulse cyclophosphamide and oral predonisolone were started. After treatment, perinuclear anti-neutrophil cytoplasmic antibodies decreased to the normal range. Retrograde double-balloon endoscopy after treatment showed ulcer scars and no ulcer.
The cause of gastrointestinal involvement in microscopic polyangiitis is ischemia due to vasculitis. It is difficult to diagnose small-vessel vasculitis by endoscopic biopsy. Although histological evidence of microscopic polyangiitis is important, the treatment should not be delayed by repeating the biopsy, because such delay can result in adverse sequela.
This case report shows that microscopic polyangiitis should be considered as a differential diagnosis when small intestinal changes like those in the present case are observed by endoscopy.
KeywordsMicroscopic polyangiitis Double-balloon endoscopy Small intestinal involvement ANCA-associated vasculitides
Microscopic polyangiitis (MPA) is pauci-immune, necrotizing vasculitis of small vessels without necrotizing granuloma. MPA is one of several systemic anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides, along with granulomatosis with polyangiitis and allergic granulomatous angiitis. The term MPA was advocated at the Chapel Hill International Consensus Conference in 1994 . MPA involves many organs or systems, including the skin, muscle, lung, kidney, brain, heart, eye, gastrointestinal tract, and peripheral nervous system. According to an analysis of several retrospective European patient cohorts , MPA typically affected male patients >50 years of age in most series (female:male ratio of approximately 1:1.5). Among affected organs and systems, kidney involvement is highest (79%–100%), and gastrointestinal involvement occurs in 30% to 50% of patients . Although colonic involvement of MPA observed by endoscopy has been reported [3, 4], small intestinal involvement observed by double-balloon endoscopy (DBE) has not been reported so far. We herein report small intestinal involvement of MPA detected and followed up by DBE.
Gastrointestinal involvement is common in systemic necrotizing vasculitides, including ANCA-associated vasculitides, polyarteritis nodosa, and rheumatoid arthritis-associated vasculitis . Although gastrointestinal involvement is not rare, it is difficult to diagnose MPA by endoscopic biopsy because it affects small vessels, and endoscopic biopsy specimens are often too small and superficial for a definitive diagnosis. Watts et al.  proposed a stepwise algorithm for classification of ANCA-associated vasculitides and polyarteritis nodosa. In this algorithm, in addition to the American College of Rheumatology criteria , the Chapel Hill Consensus Conference definitions , and the Lanham criteria , ANCA and surrogate markers for vasculitis are used because histological data are not available for all patients. Even if there is no histological evidence of necrotizing vasculitis, treatment should be performed when ANCA-associated vasculitides are strongly suspected clinically. Although histological evidence of vasculitis is important, repeat biopsy should be avoided because it can result in delayed initiation of treatment and adverse sequela.
Differential diagnoses of MPA include polyarteritis nodosa, other ANCA-associated vasculitides, collagen disease (such as systemic lupus erythematosus and rheumatoid arthritis), anaphylactoid purpura, and so on. We strongly suspected ANCA-associated vasculitides based on the nerve biopsy findings, clinical symptoms, and blood test results. Signs and symptoms of upper airway involvement, granulomatous formation, asthma, and eosinophilia did not exist. Therefore, MPA was diagnosed with Watts’ algorithm.
In this case, DBE showed multiple irregular ulcerations with redness and edema in the ileum. Ulcer formation was variable. Gastrointestinal involvement of MPA is caused by ischemia due to vasculitis. Endoscopically observed small intestinal involvement in ANCA-associated vasculitides is extremely rare, and only three previous reports of this disorder as observed by endoscopy have been documented [9–11]. Among them, two cases were allergic granulomatous angiitis and one was granulomatosis with polyangiitis. To the best of our knowledge, this is the first case report of MPA complicated with small intestinal involvement detected and followed up by DBE to be documented in the world literature. DBE was an appropriate investigation in this case, because it enabled visualization of the small intestinal lesions. Although it is possible that the ileal lesion could have been observed by colonoscopy, this is by no means certain. It seemed that it would not be possible to reach the stenosis during colonoscopy. The lesions could have been visualized by capsule endoscopy. However, the risk of retention was extremely high because of the stenosis. The patient had severe mononeuritis multiplex and intestinal involvement. We classified her condition as early systemic type, which is one of the categories advocated by the European Vasculitis Study Group . However, her neuropathy was severe, so we started intravenous pulse cyclophosphamide and oral prednisolone. We were able to confirm healing of the intestinal involvement by DBE after treatment. This case report shows that more frequent disorders, such as Crohn’s disease, infections, malignant lymphoma, and drug-induced enteritis, should be suspected first, but that MPA should be considered as a differential diagnosis when small intestinal changes like those observed in the present study are observed by endoscopy.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Series Editor of this journal.
Anti-neutrophil cytoplasmic antibody
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