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A 64-year-old Finnish man was admitted to hospital because of abdominal pain and macroscopic haematuria. The pain, located in the right upper quadrant, had been continuous for 48 h. On physical examination, the findings of pulmonary and cardiac auscultation were normal. No peripheral oedema, skin rash or neurological signs were observed. His abdomen was soft, but tender, on the upper right quadrant. Findings of rectal palpation, bowel sounds and chest radiograph were normal. The body temperature was 37.8°C, and blood pressure was 146/96 mmHg. The medical records revealed a diagnosis of asthma 15 years earlier and a nasal polypectomy 2 years earlier.
The laboratory results showed elevated plasma C reactive protein (80–230 mg/l) and creatinine (267 μmol/l). Haemoglobin level (146 g/l) was normal, but a blood count indicated leucocytosis (14.0×109/l). Blood eosinophil count was elevated (0.50×109/l; normal range 0.05×109/l to 0.44×109/l). Platelet and erythrocyte counts were in the normal range (237×109/l and 4.8×1012/l, respectively). Liver function tests (plasma aminotransferases, alkaline phosphatase and bilirubin) and plasma amylase level were also normal. Serum anti-neutrophil cytoplasmic antibody titre and anti-proteinase 3 antibodies were in the normal range. However, serum perinuclear-staining anti-neutrophil cytoplasmic antibody titre was slightly elevated (320) together with substantially elevated serum antibodies for myeloperoxide (150 EU; normal range <10 EU) and serum IgE (658 kU/l; normal range <100 kU/l). Urine sample revealed microscopic haematuria and mild proteinuria (24 h urine protein ad 270 mg).
Abdominal ultrasonography revealed a solitary calculus in gall bladder, but no dilatation of biliary tracts. The liver was interpreted as normal and no ascites was found. The intravenous administration of cefuroxime and metronidazole was initiated, and the next day a laparoscopy was performed. The preliminary preoperative diagnoses were cholecystitis and/or appendicitis.
During laparoscopy an inflamed appendix, situated next to gall bladder in the right upper quadrant, was observed. The gallbladder seemed to be macroscopically normal except for a calculus. The result of intraoperative cholangiography was normal. However, the surface of the liver was covered with greyish nodules with a diameter of 1–2 mm. Appendicectomy and cholecystectomy were performed, and a liver biopsy was taken.
Histopathological examination of the resected appendix and gallbladder revealed totally obliterated vessels with active inflammation. The inflammatory cells consisted of eosinophilic and neutrophilic granulocytes, and histiocytes to a lesser extent. No giant cells were observed, but histiocytes formed a perivascular granulomatous reaction. The inflammation extended to the perivascular tissue (figure 1C–F). Otherwise the mucosa and other layers of the appendix were normal, but there was a mild lymphocytic infiltrate and focal eosinophilic accumulation in the lamina propria of the gall bladder. In the liver biopsy, a similar inflammatory vascular reaction was observed in the perilobular vessels (figure 1A). The liver parenchyma showed a mild macrovesicular steatosis, but it was otherwise normal (figure 1B). The histopathological diagnosis of Churg–Strauss vasculitis (Churg–Strauss syndrome, CSS) affecting the appendix, gall bladder and liver was established. The renal insufficiency was interpreted as an involvement of systemic vasculitis. The clinical diagnosis of CSS was made on the basis of the criteria of The American College of Rheumatology. Oral azathioprine and prednisolone treatments were initiated.
Low-power and high-power views of dense eosinophilic infiltrates and fibrinoid necrosis of small-size and medium-size vessels in the liver portal triads (A,B; H&E ×100 and ×400, respectively), subserosal layer of the gall bladder (C,D; H&E ×40 and ×400, respectively), and in the distal part of the appendix (E,F; H&E ×20 and ×400, respectively). The perivascular granulomatous reaction is illustrated in C–F. In addition to vasculitis, there is fibrous obliteration of the appendiceal lumen (E).
Discussion
CSS is a rare disease (roughly one in a million) characterised by pulmonary and systemic small-vessel necrotising vasculitis and peripheral eosinophilia occurring in individuals with asthma and often allergic rhinitis or sinusal polyposis.1 Histologically there is a small vessel necrotising vasculitis accompanied by eosinophils and granulomas. Perinuclear anti-neutrophil cytoplasmic antibodies are present in less than half of the cases. The major associations are palpable cutaneous purpura, gastrointestinal tract bleeding and renal involvement.
The signs of acute abdominal catastrophe are unusual but diagnostically important since the outcome may be lethal. Typically the patients are middle aged, but CSS with abdominal complications has been described in children2 and adolescents.3 The gastrointestinal manifestations include diarrhoea, melena and abdominal cramps.3 Rare cases of intestinal erosion,4 ulceration and perforation3 7 8 have been described. Others include acalculous cholecystitis associated with large liver abscesses,4 liver infarction with cutaneous necrosis,5 and duodenitis.6 One case of oophoritis, complicating interpretation of the abdominal signs in women, has also been documented.9
The patient in this report had a systemic vasculitis that culminated with the signs of an acute abdomen. The clinical diagnoses of acute appendicitis and/or cholecystitis were established. Laparoscopy revealed an inflamed appendix. The gall bladder seemed macroscopically uninflamed. Surprisingly, the surface of liver was totally covered with small greyish nodules indicating a disseminated neoplasm or infectious process involving microabscesses (eg, pyogenic bacterial, protozoan infection or miliary tuberculosis). Thus the vasculitis was not clinically apparent.
After the examination of the histological samples, a diagnosis of granulomatous small-vessel necrotising vasculitis accompanied with eosinophils and neutrophils was established. Total remission was achieved by steroid and azathioprine therapy. The differential diagnoses include microscopic polyangiitis, Wegener granulomatosis and polyarteritis nodosa. CSS is characteristically accompanied by granulomas and eosinophils. Wegener granulomatosis and polyarteritis nodosa are not associated with positive perinuclear-staining anti-neutrophil cytoplasmic antibody titre, and Wegener granulomatosis does not involve gastrointestinal symptoms. Microscopic polyangiitis is associated with elevated perinuclear-staining anti-neutrophil cytoplasmic antibody and anti-myeloperoxidase titres and can involve the gastrointestinal tract similar to CSS. However, the vascular reaction is leukocytoclastic and no history with asthma is involved.
We have presented a case of CSS in a middle-aged man who presented with gastrointestinal symptoms leading to appendicectomy and cholecystectomy. In addition to an inflamed gall bladder and appendix, the surface of liver was covered with nodules suspicious of a disseminated malignancy or infectious process. The diagnosis of CSS was established after histopathological examination and serological tests. To our knowledge, this type of potentially confusing clinical manifestation of the CSS has not been reported previously.
Take-home messages
Churg–Strauss syndrome is a rare systemic small-vessel necrotising vasculitis, which may infrequently be manifested by symptoms of an acute abdomen.
Careful evaluation of patient history (asthma, nasal polyposis, allergic rhinitis), signs of systemic disease (renal, cardiac, neurological, dermatological) and haematological findings (eosinophilia, elevated serum IgE and perinuclear-staining anti-neutrophil cytoplasmic antibodies) are needed.
Histopathological verification of small-vessel necrotising vasculitis accompanied by eosinophils and granulomas is essential for definitive diagnosis.
Footnotes
Competing interests None declared.
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