A rare cause of ischemic colitis: Polyarteritis nodosa

Summary This paper details the first case report of a patient with fulminant, gangrenous, ischemic colitis caused by polyarteritis nodosa which was successfully treated surgically. Ischemic colitis is a rare complication of polyarteritis nodosa. It should be suspected in patients with a past history of polyarteritis nodosa who develop abdominal pain and rectal bleeding. The identification of cutaneous lesions preoperatively and or mesenteric or serosal vascular beading at operation are helpful in establishing this diagnosis. Prognosis is generally determined by the extent of systemic involvement by polyarteritis nodosa, and death is generally associated with renal failure.     

Abdominal aApoplexy in polyarteritis nodosa

Summary A case of intraperitoneal hemorrhage due to the rupture of an aneurysm of the middle colic artery in a patient with polyarteritis nodosa is described. The importance of early surgical intervention in patients with polyarteritis nodosa who develop sudden abdominal pain is emphasized. Boston City Hospital Boston, Mass. 02118Research Fellow in Medicine, Harvard Medical School and Thorndike Memorial Laboratory.     

Polyarteritis nodosa: ischaemic intestinal pain successfully treated with nitroglycerin

Inflammation of medium and small sized arteries caused by polyarteritis nodosa (PAN) sometimes causes acute and life threatening ischaemic intestinal complications. We report a case of PAN presenting with severe abdominal pain successfully treated with nitroglycerin.     

A case of polyarteritis nodosa with intra-abdominal bleeding due to rupture of the hepatic aneurysm]

A 39-year-old woman was admitted to our hospital because of abdominal pain. She was diagnosed as intra-abdominal bleeding and an emergency laparotomy was performed. On laparotomy, massive bleeding in the abdominal cavity due to a ruptured aneurysm of the intrahepatic artery was found. We also verified small aneurysm of the common hepatic artery, tinged with red, and was suspected systemic vasculitis. The post-operative course was uneventful, but the subsequent angiography revealed many other small aneurysm of hepatic, renal and lumbar aytery. Then it was diagnosed as polyarteritis nodosa. A case of polyarteritis nodosa presenting with intra-abdominal homorrhage like this case is rare, so we presented here together with a review of the literature.     

Duodenal necrosis as the presenting manifestation of polyarteritis nodosa

Polyarteritis nodosa involves necrotising vasculitis of small and medium-sized arteries. Multiple organ systems are involved. A non-specific and slow course of disease is common. Gastrointestinal involvement is characterised by abdominal pain, nausea and vomiting. Bowel infarction and perforation, cholecystitis and hepatic infarction are well known complications. However, bowel infarction as the presenting symptom of the disease is rare. The case of a 20-year-old male with necrosis of the duodenum heralding polyarteritis nodosa is reported. The patient made a slow recovery after extensive abdominal surgery and a stormy course. The postoperative management and treatment of polyarteritis nodosa are discussed. A high index of suspicion and prompt multidisciplinary approach are needed in order to improve survival in these rare but potentially fatal conditions. Received: 15 May 2001 / Accepted: 5 February 2002     

Penetrating colon ulcer of polyarteritis nodosa: report of a case

A 54-year-old Japanese female with polyarteritis nodosa was admitted to the hospital. She developed lower abdominal pain accompanied by melena. A penetrating ulcer and extensive hemorrhaging were endoscopically observed in the sigmoid colon, and a sigmoidectomy was performed. The pathologic findings were a granuloma formation with lymphocytic infiltration and luminal occlusion of branches of the mesenteric arteries. Although the gastrointestinal tract is frequently involved in polyarteritis nodosa, the colon is rarely affected. To our knowledge, this is the first report of polyarteritis nodosa causing a penetrating ulcer of the colon.     

Polyarteritis nodosa and Henoch–Schönlein purpura nephritis in a child with familial Mediterranean fever: a case report

Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Approximately 5% of individuals with familial Mediterranean fever have been reported to have Henoch–Schonlein purpura and about 1% to have polyarteritis nodosa. A 7-year-old girl presenting with complaints of purpuric rash, abdominal pain, arthritis, hematuria, and proteinuria and having IgA depositions on renal biopsy was diagnosed as Henoch–Schönlein nephritis. She had a history of recurrent fever, abdominal and joint pain and M694 V compound homozygote mutation. Colchicine treatment was started for the diagnosis of FMF. When constitutional symptoms such as myalgia, weight loss, fatigue, fever, and hypertension were added to the clinical picture, the diagnosis of polyarteritis nodosa HSP was thought and confirmed by the demonstration of microaneurisms on renal arteries. There was no response to corticosteroid and cyclophosphamide treatments; however, the symptoms were rapidly and dramatically reduced after the administration of intravenous immunoglobulin. In conclusion, polyarteritis nodosa and Henoch–Schonlein purpura can be seen together with familial Mediterranean fever. It is also suggested that IVIG might be an important adjunct therapy in selected patients with polyarteritis nodosa, especially in the lack of response to steroids and immunsuppressive drugs.     

A case of ischemic colitis in a patient with Takayasu's arteritis]

Ischemic colitis is one of the most common intestinal ischemic injury in which more than 90% of patient are over 60 year-old. It results from impaired perfusion of blood to the bowel and is rarely caused by vasculitis such as systemic lupus erythematosus, polyarteritis nodosa, and Takayasu's arteritis. Takayasu's arteritis affects the aortic arch, medium-sized and large arteries but rarely involves inferior mesenteric artery. We report a case of Takayasu's arteritis involving inferior mesenteric artery which developed ischemic colitis in a 70 year old female. To the author's knowledge this is the first case report in Korea. A 70 year old woman who had suffered from Takayasu's arteritis for 5 years was admitted for sudden abdominal pain and hematochezia. On sigmoidoscopy, there were multiple segmental longitudinal ulcerations around splenic flexure and diffuse hemorrhagic edematous mucosa from descending colon to sigmoid colon. On abdominal CT angiography, inferior mesenteric artery was not traced. We diagnosed it as ischemic colitis combined with Takayasu's arteritis. After the conservative treatment, abdominal pain and hematochezia disappeared. She was followed up to 2 years without recurrence of symptoms.     

Intestinal ischamemia in a child due to polyarteritis nodosa: a case report.

Polyarteritis nodosa (PAN) is a rare type of childhood vasculitis. We report the case of a 10-year-old boy who was referred to our hospital following an appendicectomy with fever, abdominal pain, vomiting, diarrhoea, weight loss, joint pains and skin rash. Shortly after admission, he developed peritonitis, and an emergency laparotomy revealed ischaemia of the jejunum which required partial resection. Histologic examination of the resected bowel showed features consistent with PAN. He was treated with steroids and cyclophosphamide. Nineteen months later, he is asymptomatic and thriving. PAN should be included in the differential diagnosis if a child with constitutional symptoms also has gastrointestinal complaints or develops an acute abdomen.     

A young female case of polyarteritis nodosa strongly suspected by typical angiographic findings which improved rapidly after prednisolone and cyclophosphamide therapy.

A 16-year-old girl was admitted with the complaints of headache, chest pain, low abdominal pain and left hemi-numbness. Her blood pressure was high and plasma renin activity and aldosterone levels were elevated. Renal angiography revealed vascular stenoses and microaneurysms although the renal artery and its main branches were not involved. Polyarteritis nodosa (PN) was strongly suspected and oral prednisolone and intravenous pulse therapy of cyclophosphamide were started. The second renal angiography which was performed 11 days after the therapy was started, showed marked improvement of vascular lesions. This is a case which suggests that the angiographic findings of PN can improve very rapidly with therapy.     

Hepatitis B-related polyarteritis nodosa complicated by pulmonary hemorrhage

A 34-year-old man presented with fever, weight loss, paresthesia, abdominal pain, and hypertension. He had hepatitis B antigenemia, with negative antineutrophil cytoplasmic antibody, antinuclear antibody, and antiglomerular basement membrane serology results. Renal arteriography showed multiple intrarenal microaneurysms. In spite of therapy with antiviral agents (lamivudine, famciclovir), prednisone, cyclophosphamide, and plasmapheresis, renal function deteriorated. He later developed rapidly progressive dyspnea and hemoptysis. Diffuse alveolar hemorrhage was confirmed by bronchoscopy. He died of respiratory failure. The cause of pulmonary hemorrhage in this case of polyarteritis nodosa is unclear, but may include underlying capillaritis, cocaine-induced pulmonary hemorrhage, or recurrent attacks of malignant hypertension.     

Post-infantile giant cell hepatitis associated with autoimmune hepatitis and polyarteritis nodosa

We report the case of a patient with corticosteroid-responsive giant cell hepatitis associated with typical manifestations and changes of polyarteritis nodosa from the kidney and central nervous system. Initially, the patient presented with transient right hemiparesis, followed by spontaneous remission without any abnormalities on computed tomography scan, magnetic resonance imaging and cerebrospinal fluid examination. A few months later he was admitted to our clinic because of icterus, peripheral oedema and abdominal distension. He was found to have clinical signs of active cirrhosis. Serological tests for hepatitis B, C and HIV virus were negative. Serum ceruloplasmin. a1-AT and ferritin levels were within normal limits. Antinuclear antibodies were positive (1: 160). Liver biopsy showed micronodular cirrhosis with many eosinophils in the portal tracts and giant hepatocytes with multiple nucleoli in the lobule. Fulfilling the diagnostic criteria for autoimmune hepatitis, he was started on treatment with prednisolone and azathioprine, resulting in both clinical and biochemical responses. Four years later he presented with severe pain at the right costovertebral angle. Ultrasonography revealed a haematoma at the right kidney, and selective angiography of the abdominal aorta, renal arteries and hepatic artery documented microaneurysms in both kidney and liver arteries. Because of severe haemorrhage, right nephrectomy was performed. Histology of kidney specimen showed characteristic lesions of polyarteritis nodosa. Several months later, while on treatment with prednisolone and cyclophosphamide, the patient experienced a fatal episode of brain haemorrhage. An association between autoimmune hepatitis, polyarteritis nodosa and postinfantile giant cell hepatitis has not been reported previously.     

Gastrointestinal bleeding and intestinal perforation due to polyarteritis nodosa in a patient with celiac disease. A case report

Complications of celiac disease could present with intestinal perforation and rarely, gastrointestinal bleeding, which are usually secondary to nongranulomatous ulcerative jejunoileitis or T-cell lymphoma. We describe the case of a 66-year-old male patient with an 8-year history of celiac disease (CD) who presented with recurrent abdominal pain and gastrointestinal bleeding. Several tests were performed to find out possible complications associated to CD. Due to an overt gastrointestinal bleeding, an arteriography was performed and signs of polyarteritis nodosa were found. Vascular disease was aggressive, and despite multiple medical and surgical treatments the patient died. As arteriography is not usually performed for the study of the complications of CD, it is possible that the association between CD and PAN has been underdiagnosed.     

Successful treatment of pediatric systemic polyarteritis nodosa with cholestatic hepatitis

Polyarteritis nodosa (PAN) is a rare vasculitis in children. We report a case of 5-year-old boy with systemic PAN and cholestatic hepatitis. He had fever, abdominal pain, and gastrointestinal bleeding. Angiography revealed multiple microaneurysms in the renal, hepatic, and superior mesenteric arteries. Clinical manifestations improved slowly after immunosuppressive therapy, but liver enzyme and bilirubin levels elevated gradually. Liver biopsy findings revealed marked centrizonal canalicular cholestasis, bile duct damage, and intact hepatocyte, but there was no evidence of viral hepatitis or vasculitis. Levels of liver enzymes and bilirubin improved after two cycles of cyclophosphamide therapy. We thought that the possible etiology of elevated liver enzyme and bilirubin levels might be a manifestation of PAN.     

Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients

PURPOSE: Gastrointestinal involvement in polyarteritis nodosa carries a poor prognosis. A 1982 review from our institution reported acute abdominal syndromes in 31% of patients with polyarteritis nodosa, and that all 5 patients with acute surgical abdomens died. We reviewed our more recent experience to determine if outcomes have changed since. SUBJECTS AND METHODS: We reviewed the records of all patients with polyarteritis nodosa in our vasculitis database between 1986 and 2000. Inclusion criteria were a diagnosis of polyarteritis nodosa, symptoms or signs of gastrointestinal involvement, and either a mesenteric angiogram consistent with polyarteritis nodosa or histopathologic proof of a medium-vessel vasculitis. We calculated a prognostic (5 factor) score for all patients. RESULTS: We identified 24 patients with polyarteritis nodosa who had gastrointestinal involvement during their illness. Thirteen (54%) of the patients developed acute surgical abdomens, 3 of whom died (P = 0.02 by comparison with the historical cohort). Mean (+/- SD) prognostic scores were higher among patients in the acute abdomen group compared with those who did not have acute abdominal syndromes (1.7 +/- 0.9 vs. 0.6 +/- 0.7, P = 0.002), corresponding with the observed mortality in these groups. CONCLUSION: Gastrointestinal involvement occurs commonly in polyarteritis nodosa and carries a poor prognosis. Compared with a historical cohort at our institution, mortality from this complication may have decreased, perhaps because of earlier diagnosis.     

Oedematous polyarthritis and myopericarditis as presenting features of periarteritis nodosa

Remitting symmetrical seronegative synovitis with pitting edema (RS3PE) syndrome is a rare type of seronegative polyarthritis occurring in the elderly. It can be associated to various diseases. We report a case of RS3PE syndrome associated with myopericarditis, leading to the diagnosis of polyarteritis nodosa in a 71-year-old patient admitted to the hospital for a febrile acute polyarthritis with pitting edema of the hands associated with a marked inflammatory syndrome. On second day of hospitalization, a sustained chest pain led to the diagnosis of myopericarditis. Muscular biopsy showed necrotizing vasculitis, characteristic of polyarteritis nodosa. The coexistence of RS3PE and myopericarditis has never been described in the literature. Its association with polyarteritis nodosa is also very rare and only one case has been previously reported.     

A case of acute pancreatitis caused by 5-aminosalicylic acid suppositories in a patient with ulcerative colitis]

Oral 5-aminosalicylic acid (5-ASA) has been known as a first-choice drug for ulcerative colitis. However, hypersensitivity reactions, including pancreatitis, hepatitis, and skin rash, have been reported with 5-ASA. Topical formulations of 5-ASA like suppositories have been rarely reported to induce adverse reactions because of their limited absorption rate. We recently experienced a case of acute pancreatitis caused by 5-ASA suppositories in a patient with ulcerative colitis. A 26-year-old male was admitted with abdominal pain and diagnosed as ulcerative colitis. Acute pancreatitis occurred soon after 24 hours of treatment with oral mesalazine. Drug-induced pancreatitis was suspected and administration of mesalazine was discontinued. Then 5-ASA suppositories were started instead of oral mesalazine. Twenty-four hours after taking 5-ASA suppositories, he experienced severe abdominal pain, fever, and elevation of amylase levels. The suppositories were immediately stopped and symptoms resolved over next 48 hours. Herein, we suggest that, in patients treated with 5-ASA suppositories who complain of severe abdominal pain, drug-induced pancreatitis should be suspected.     

Treatment of a patient with clinical features of both eosinophilic gastroenteritis and polyarteritis nodosa with oral sodium cromoglycate

Summary A young patient who suffered from allergic respiratory disease presented with an illness characterized by abdominal pain, diarrhea, and weight loss. Investigations revealed radiological and pathological features consistent with a diagnosis of eosinophilic gastroenteritis. Because of extensive gastric ulceration, treatment with corticosteroids was with-held and in its place oral sodium cromoglycate therapy given. The patient's intestinal symptoms rapidly responded to this treatment and remained controlled for a period of five months. The patient subsequently developed a systemic illness with features of polyarteritis nodosa.     

Necrotizing Vasculitis of the Gallbladder and the Appendix

Two patients with rheumatoid arthritis (RA) and polyarteritis nodosa (PN) presented with abdominal pain. Both revealed necrotizing vasculitis of the gallbladder and the appendix respectively. The controversial role of corticosteroids in the pathogenesis of rheumatoid arteritis, the rarity of rheumatoid arteritis of the gallbladder and the morphologic similarity of the vascular lesion in the gallbladder and appendix are discussed.     

Digestive manifestations of periarteritis nodosa in a series of 120 cases

In a series of 120 patients with periarteritis nodosa (PAN), 50 had gastrointestinal manifestations; 34 had transient abdominal pain which regressed spontaneously or in response to corticosteroid therapy and required no further investigation. Thirty one more serious episodes occurred in the remaining 26 patients. Eight of these were in fact the initial signs of PAN and 13 required laparotomy. There were 20 episodes of abdominal pain (peritonitis: 9, pancreatitis: 4, acute cholecystitis: 2, duodenal ulcer: 3, intestinal infarction: 1, unexplained pain without diagnosis at laparotomy: 1) and 11 of gastrointestinal hemorrhage (melaena or hematemesis: 4; hematochezia: 5). Clinical and biological features of patients with and without gastrointestinal manifestation were not significantly different except for cardiac involvement which was significantly more frequent (p less than 0.05) in the second group. Corrected survival rates were significantly lower (p less than 0.05) in patients with gastrointestinal manifestations. These results show that, in patients with PAN, digestive manifestations, particularly perforations, carried a poor prognosis. Nevertheless exploratory laparotomy and surgery unrelated to PAN (eg appendicectomy) were well tolerated.