(perinuclear staining pattern [p-ANCA]) was positive on one occasion but subsequently negative. She required parenteral nutrition for 11 weeks before shewas ...
JOURNAL OF THE ROYAL SOCIETY OF MEDICINE
Volume 88
December 1 995
Systemic vasculitis presenting with massive bowel infarction D A Collins MD MRCPI
0 Duke MD FRCP
J R Soc Med 1995;88:692-693
Keywords: vasculitis; bowel infarction; corticosteroids
SUMMARY Gastrointestinal involvement in systemic vasculitis occurs in up to 30% of patients. Fortunately intestinal infarction is a rare complication, but if present carries a high mortality, and swift management of the underlying vasculitis is crucial. Two such cases are described.
CASE I A 48-year-old woman was admitted for investigation of a 12 week history of sore throat, fever and myalgias, and 6 weeks of arthralgias, and arthritis of her ankles, wrists, metacarpophalangeal joints and shoulders, with night sweats and 10kg weight loss. She had no rash, cough, breathlessness or gastrointestinal symptoms; no other relevant medical history and was on no medication. On admission she was pyrexial (37.6°C) with a tachycardia and synovitis of wrists and ankles. There was no rash or lymphadenopathy. Her abdomen was soft but tender in the epigastrium. Investigations revealed Hb 10.2 g/dl, white cell count (WCC) 15.6 x 109/l, platelets 697 x 109/l, ESR 108 mm in the first hour. Serum albumin 25 g/l, C-reactive protein 315 mg!/ (normal < 10). Thirty-six hours after admission she developed abdominal pain and vomiting, a rigid abdomen and absent bowel sounds. On the assumption that she had a connective tissue disease intravenous methylprednisolone was given and a laparotomy was performed, revealing large areas of gangrenous small and large bowel. She required a partial gastrectomy, total colectomy and resection of all but 60 cm of proximal jejunum, and 60 cm of terminal ileum. Histology showed necrotizing vasculitis of small and medium sized arteries without granulomata, compatible with polyarteritis nodosum (PAN). Antinuclear antibodies (ANA), rheumatoid factor, and hepatitis B serology were negative, and antineutrophil cytoplasmic antibodies (perinuclear staining pattern [p-ANCA]) was positive on one occasion but subsequently negative. She required parenteral nutrition for 11 weeks before she was able to convert to a supplemented oral diet. During this time she was treated with pulsed intravenous cyclophosphamide and
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Department of Rheumatology, St Helier Hospital, Carshalton, Surrey, UK Correspondence to: Dr D A Collins, Department of Rheumatology, St George's Hospital, London SW17 OQT, UK
methylprednisolone and suffered no further problems. She was able to return home after 20 weeks and remains well on treatment. CASE 2 A 39-year-old man was admitted for consideration of lobectomy, having presented with a 6 week history of chest pains, arthralgias and bone pains, and on chest X-ray a peripheral mass in the upper lobe of his left lung. Computerized tomography showed this to be a cavitating lesion. Bronchoscopy showed no abnormality but cytology suggested a squamous cell carcinoma. He was previously healthy with no other symptoms, had no relevant past medical history. He was on no medication but was an exsmoker of 20 cigarettes/day. Examination was unremarkable. Full blood count, liver and renal function was normal. He deteriorated 48 h before admission with breathlessness and abdominal pain. On examination he was pale, pyrexial (38.20C) and cyanosed. He was tender over the right iliac fossa. Investigations revealed renal failure (urea 44mmol/l, creatinine 815gjmol/l), Hb 11.7g/dl, WCC 28.8 x 109/l, Pts 357 x 109/l. Abdominal ultrasound was normal. He developed increasing abdominal pain and peritonitis and was transferred to ITU. Seventy-two hours after admission laparotomy revealed an infarcted and necrotic perforated colon. Colectomy was performed. Twenty-four hours later he was noted to have infarcted lesions on his fingertips. The following day an open biopsy of the lung lesion was performed. Histology from the infarcted bowel and lung showed a necrotising granulomatous vasculitis compatible with Wegener's granulomatosis. A retrospective serum assay revealed him to be c-ANCA positive. Despite immunosuppression as soon as the histology was available (5 days after admission) with methylprednisolone and later cyclophosphamide, he required further bowel resection and
JOURNAL OF THE ROYAL SOCIETY OF. MEDICINE
progressed into multisystem failure with recurrent sepsis, and died 9 days later. DISCUSSION
Mesenteric vasculitis is a rare but potentially serious complication of systemic vasculitis. It is reported in association with rheumatoid arthritis, systemic lupus erythematosus, scleroderma, PAN, Churg-Strauss vasculitis, Wegener's granulomatosis and giant-cell arteritis. Typical features are of diffuse non-specific abdominal pain progressing on occasion to gastrointestinal haemorrhage, perforation or more rarely infarction. Abdominal pain occurs in about 30% of most reported series of known vasculitis. Guillevin et al.1 reported 52 out of 165 patients with PAN or Churg-Strauss vasculitis as having abdominal symptoms, although they only found one case of bowel infarction. Gastrointestinal disease was the most common cause of immediate death in their five-year follow up study, and age greater than 50 and abdominal signs were the two factors most associated with a poor prognosis. Lopez et al.2 found gastrointestinal symptoms in 36% of 106 patients with vasculitis but intestinal infarction in only three out of 13 patients with PAN. Scott et al.3 noted abdominal pain as a feature in 30% of 80 patients with systemic vasculitis rising to 78% in the PAN group, where all patients with intestinal infarction died. The risk of death is greatest in the first 6 months after presentation. In both these cases the diagnosis of vasculitis was not made before the development of a surgical emergency. However, both cases had features suggesting systemic inflammation as a prodrome to their presentation and in Case I the correct diagnosis was guessed and steroids given immediately, which may have influenced the eventual good outcome. These patients often present as surgical emergencies4; in the case of haemorrhage or pain laparotomy is not immediately necessary, whereas presentation with perforation or severe peritonitis will usually require surgical intervention. Cyclophosphamide in combination with corticosteroids is now accepted as the treatment of choice for induction of
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December 1995
remission in systemic vasculitis5'6. In the presence of intraabdominal sepsis and recent surgery, however, this is not without risk, yet should not be shied away from as treatment of the underlying vasculitis remains imperative. Treatments with plasma exchange may also be of benefit7 but there is no controlled data on its use in the absence of accompanying corticosteroids. In patients with known or suspected systemic vasculitis (especially PAN) mesenteric involvement carries a poor prognosis. Patients with known active disease should be referred to the appropriate local specialist (usually a rheumatologist or renal physician) for urgent investigation and early institution of aggressive immunosuppression, if appropriate.. If needed this should not be deferred in the presence of an acute surgical emergency. Acknowledgments We are grateful to Professor Tom Treasure for permission to report Case No. 2. REFERENCES 1 Guillevin L, Huong DTL, Godern P, Jais P, Wechsler B. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study of1.165 patients. BrJ Rheumatol 1988;27:258-64 2 Lopez LR, Schocket AL, Standford RE, Claman HN, Kohler PF. Gastrointestinal involvement in leucocytoclastic vasculitis and polyarteritis nodosa. J Rheumatol 1980;7:677-84 3 Scott DGI, Bacon PA, Elliott PJ, Tribe CR, Wallington TB. Systemic vasculitis in a district general hospital 1972-1980: Clinical and laboratory features, classification and prognosis of 80 cases. QJ Med 1982;203:292311 4 Colton CL, Butler TJ. The surgical problem of polyarteritis nodosa. BrJ Sur 1967;54:393-6 5 Fauci AS, Katz P, Haynes BF, Wolff SM. Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Englj Med 1979;301:235-8 6 Scott DGI, Bacon PA. Intravenous cyclophosphamide and methylprednisolone in the treatment of systemic rheumatoid vasculitis. Am J Med 1984;76:377-84 7 Guillevin LO, Jarrouse B, Lok C, et al. Long term follow-up after treatment of polyarteritis nodosa and Churg-Strauss angiitis, with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. J Rheumatol 1991 ;18:567-74
(Accepted 9 June 1995)
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