1991, The British Journal of Radiology, 64, 959-960
Case reports Small bowel vasculitis By F. V. Gleeson, MRCP, FRCR and *P. J. Tidbury, MB, BS Departments of Radiology and 'Pathology, Whittington Hospital, Highgate Hill, London N19 5NF, UK {Received December 1990 and in revised form March 1991)
Keywords: Small bowel, Vasculitis
Systemic vasculitis affecting the gastrointestinal tract is uncommon. We report a case affecting only the small bowel. The gastrointestinal manifestations and diagnosis of vasculitis are discussed. Case report A 55-year-old normotensive man with a past history of a left nephrectomy in 1970 for nephrolithiasis, presented with a 3-month history of upper abdominal pain after food, which occasionally radiated through to his back. The pain lasted up to 2 hours, and was sometimes relieved by vomiting. There was no history of haematemesis or melaena. Initial haematological investigation was normal and endoscopy demonstrated only mild oesophagitis. Treatment with cimetidine failed to relieve his symptoms. Ultrasound demonstrated a thick-walled gallbladder containing a gallstone, but following cholecystectomy his pain persisted. On follow-up he became anorexic and had lost 1 stone in weight. He was readmitted and a repeat gastroscopy revealed multiple umbilicated ulcers in the gastric antrum and proximal duodenum. The patient was now pyrexial and obviously unwell, he was anaemic, had a raised white cell count, and an elevated alkaline phosphatase level. Small bowel lymphoma was suspected and a barium follow through performed. The follow through demonstrated markedly abnormal small bowel, with strictures, dilated small bowel loops, areas of "picket fencing", a "stacked coin appearance" and a featureless collection of barium in the left hypochondrium (Fig. 1). Lymphoma or serosal deposits from an unknown primary were considered the most likely radiological diagnosis. Unfortunately, 2 days after the barium examination the patient became shocked and attempted resuscitation proved unsuccessful. At post-mortem examination the small bowel was found to be ulcerated and strictured, with a sealed perforation in the left hypochondrium. The mesenteric vessels and their origins were normal. Microscopic examination of the small bowel wall demonstrated the changes of polyarteritis nodosa (Fig. 2). There was no evidence of vasculitic involvement of his solitary kidney or elsewhere.
Discussion
The vasculitic syndromes are a heterogeneous group of diseases with the common denominator being an Address correspondence to F. V. Gleeson, Radiology Department, Royal Free Hospital, Pond Street, London NW3 2QG, UK. Vol. 64, No. 766
inflammatory and sometimes necrotic process of the blood vessel walls (Fauci et al, 1978). The size, type and location of vessels involved vary within the syndromes, and it is these variations that are responsible for the different clinical presentations (Anonymous, 1985). The vasculitic process may be the sole manifestation of the disease or may be a part of another underlying primary condition such as systemic lupus erythematosus. The clinical manifestations of gastrointestinal vasculitis are usually abdominal pain, diarrhoea or rectal bleeding, and may prove difficult to differentiate from inflammatory bowel disease, intestinal ischaemia or infection (Camilleri et al, 1983). The changes, like those due to vasculitis elsewhere, are the result of local ischaemia and may result in mucosal ulceration, local areas of gangrene with perforation and peritonitis, large areas of gangrene with stricture formation and obstruction, and haemorrhage (Colton & Butler, 1967). Historically, polyarteritis nodosa is characterized by involvement of predominantly medium-sized arteries. These show fibrinoid necrosis of the arterial wall, inflammatory reaction in and around the artery, aneurysm formation and thrombosis of the lumen. Involvement is often segmental and there may be associated areas of healing. In reported series, gastrointestinal involvement occurred only in the presence of other clinical evidence of a systemic vasculitis (Camilleri et al, 1983), and the frequency is dependent on the type of vasculitis, with the highest incidence occurring in cases of polyarteritis nodosa (Matolo & Albo, 1971). The diagnosis of vasculitis affecting the gut is often dependent on evidence of clinical involvement elsewhere. Visceral angiography may demonstrate aneurysms in up to 60% of patients with polyarteritis nodosa (Travers et al, 1979). Plain abdominal radiographs and barium studies, as in this case, may be abnormal, showing dilated small bowel with normal or thickened folds, although the changes are not diagnostic (Eisenberg, 1990). Histological proof of vasculitis is difficult to obtain from biopsy and is often only possible from resection of macroscopically abnormal bowel (Camilleri et al, 1983). Haematological evidence of vasculitis may be provided by the detection of anti-neutrophil cytoplasmic antibodies (Savage et al, 1987); this test was not performed in our case. 959
Case reports
(a)
(b)
Figure 1. (a, b) Barium follow-through study demonstrating the sealed perforation in the left hypochondrium (curved arrow) and the "picket-fencing" (straight arrows) and "stacked-coin" appearance in the left iliac fossa.
We believe this case to be important as only the small bowel was affected, with no evidence at autopsy of involvement elsewhere. It also serves as a reminder of the difficulty in making the diagnosis of a vasculitis.
References ANONYMOUS, 1985. Systemic vasculitis (Editorial). Lancet, i, 1252-1254. CAMILLERI, M., PUSEY, C. D., CHADWICK, V. S. & REES, A. J.,
1983. Gastrointestinal manifestations of systemic vasculitis. Quarterly Journal of Medicine, 206, 141-149. COLTON, C. L. & BUTLER, T. J., 1967. The surgical problem of
polyarteritis nodosa. British Journal of Surgery, 54, 393396. EISENBERG, R. L., 1990. Gastrointestinal Radiology. A Pattern Approach (J. B. Lippincott, Philadelphia), pp. 450, 458. FAUCI, A. S., HAYNES, B. F. & KATZ, P., 1978. The spectrum of
vasculitis. Clinical, pathological, immunologic and therapeutic considerations. Annals of Internal Medicine 89 660-676. MATOLO, N. M. & ALBO, D., J R , 1971. Gastrointestinal
complications of collagen vascular diseases. Surgical implications. American Journal of Surgery, 122, 678-682. SAVAGE, C. O. S., WINEARLS, C. G., JONES, S., MARSHALL,
Figure 2. Elastic Van-Gieson-stained histological section showing a small artery with destruction of the elastic lamina and media with aneurysm formation. The aneurysm is filled with organizing thrombus.
960
P. D. & LOCKWOOD, C. M , 1987. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis. Lancet, i, 1389-1393. TRAVERS, R. L., ALLISON, D. J., BRETTLE, R. P. & HUGHES,
G. R. V., 1979. Polyarteritis nodosa: clinical and angiographic analysis of 17 cases. Seminars in Arthritis & Rheumatology, 8, 184-198.
The British Journal of Radiology, October 1991
Case reports Small bowel vasculitis By F. V. Gleeson, MRCP, FRCR and *P. J. Tidbury, MB, BS Departments of Radiology and 'Pathology, Whittington Hospital, Highgate Hill, London N19 5NF, UK {Received December 1990 and in revised form March 1991)
Keywords: Small bowel, Vasculitis
Systemic vasculitis affecting the gastrointestinal tract is uncommon. We report a case affecting only the small bowel. The gastrointestinal manifestations and diagnosis of vasculitis are discussed. Case report A 55-year-old normotensive man with a past history of a left nephrectomy in 1970 for nephrolithiasis, presented with a 3-month history of upper abdominal pain after food, which occasionally radiated through to his back. The pain lasted up to 2 hours, and was sometimes relieved by vomiting. There was no history of haematemesis or melaena. Initial haematological investigation was normal and endoscopy demonstrated only mild oesophagitis. Treatment with cimetidine failed to relieve his symptoms. Ultrasound demonstrated a thick-walled gallbladder containing a gallstone, but following cholecystectomy his pain persisted. On follow-up he became anorexic and had lost 1 stone in weight. He was readmitted and a repeat gastroscopy revealed multiple umbilicated ulcers in the gastric antrum and proximal duodenum. The patient was now pyrexial and obviously unwell, he was anaemic, had a raised white cell count, and an elevated alkaline phosphatase level. Small bowel lymphoma was suspected and a barium follow through performed. The follow through demonstrated markedly abnormal small bowel, with strictures, dilated small bowel loops, areas of "picket fencing", a "stacked coin appearance" and a featureless collection of barium in the left hypochondrium (Fig. 1). Lymphoma or serosal deposits from an unknown primary were considered the most likely radiological diagnosis. Unfortunately, 2 days after the barium examination the patient became shocked and attempted resuscitation proved unsuccessful. At post-mortem examination the small bowel was found to be ulcerated and strictured, with a sealed perforation in the left hypochondrium. The mesenteric vessels and their origins were normal. Microscopic examination of the small bowel wall demonstrated the changes of polyarteritis nodosa (Fig. 2). There was no evidence of vasculitic involvement of his solitary kidney or elsewhere.
Discussion
The vasculitic syndromes are a heterogeneous group of diseases with the common denominator being an Address correspondence to F. V. Gleeson, Radiology Department, Royal Free Hospital, Pond Street, London NW3 2QG, UK. Vol. 64, No. 766
inflammatory and sometimes necrotic process of the blood vessel walls (Fauci et al, 1978). The size, type and location of vessels involved vary within the syndromes, and it is these variations that are responsible for the different clinical presentations (Anonymous, 1985). The vasculitic process may be the sole manifestation of the disease or may be a part of another underlying primary condition such as systemic lupus erythematosus. The clinical manifestations of gastrointestinal vasculitis are usually abdominal pain, diarrhoea or rectal bleeding, and may prove difficult to differentiate from inflammatory bowel disease, intestinal ischaemia or infection (Camilleri et al, 1983). The changes, like those due to vasculitis elsewhere, are the result of local ischaemia and may result in mucosal ulceration, local areas of gangrene with perforation and peritonitis, large areas of gangrene with stricture formation and obstruction, and haemorrhage (Colton & Butler, 1967). Historically, polyarteritis nodosa is characterized by involvement of predominantly medium-sized arteries. These show fibrinoid necrosis of the arterial wall, inflammatory reaction in and around the artery, aneurysm formation and thrombosis of the lumen. Involvement is often segmental and there may be associated areas of healing. In reported series, gastrointestinal involvement occurred only in the presence of other clinical evidence of a systemic vasculitis (Camilleri et al, 1983), and the frequency is dependent on the type of vasculitis, with the highest incidence occurring in cases of polyarteritis nodosa (Matolo & Albo, 1971). The diagnosis of vasculitis affecting the gut is often dependent on evidence of clinical involvement elsewhere. Visceral angiography may demonstrate aneurysms in up to 60% of patients with polyarteritis nodosa (Travers et al, 1979). Plain abdominal radiographs and barium studies, as in this case, may be abnormal, showing dilated small bowel with normal or thickened folds, although the changes are not diagnostic (Eisenberg, 1990). Histological proof of vasculitis is difficult to obtain from biopsy and is often only possible from resection of macroscopically abnormal bowel (Camilleri et al, 1983). Haematological evidence of vasculitis may be provided by the detection of anti-neutrophil cytoplasmic antibodies (Savage et al, 1987); this test was not performed in our case. 959
Case reports
(a)
(b)
Figure 1. (a, b) Barium follow-through study demonstrating the sealed perforation in the left hypochondrium (curved arrow) and the "picket-fencing" (straight arrows) and "stacked-coin" appearance in the left iliac fossa.
We believe this case to be important as only the small bowel was affected, with no evidence at autopsy of involvement elsewhere. It also serves as a reminder of the difficulty in making the diagnosis of a vasculitis.
References ANONYMOUS, 1985. Systemic vasculitis (Editorial). Lancet, i, 1252-1254. CAMILLERI, M., PUSEY, C. D., CHADWICK, V. S. & REES, A. J.,
1983. Gastrointestinal manifestations of systemic vasculitis. Quarterly Journal of Medicine, 206, 141-149. COLTON, C. L. & BUTLER, T. J., 1967. The surgical problem of
polyarteritis nodosa. British Journal of Surgery, 54, 393396. EISENBERG, R. L., 1990. Gastrointestinal Radiology. A Pattern Approach (J. B. Lippincott, Philadelphia), pp. 450, 458. FAUCI, A. S., HAYNES, B. F. & KATZ, P., 1978. The spectrum of
vasculitis. Clinical, pathological, immunologic and therapeutic considerations. Annals of Internal Medicine 89 660-676. MATOLO, N. M. & ALBO, D., J R , 1971. Gastrointestinal
complications of collagen vascular diseases. Surgical implications. American Journal of Surgery, 122, 678-682. SAVAGE, C. O. S., WINEARLS, C. G., JONES, S., MARSHALL,
Figure 2. Elastic Van-Gieson-stained histological section showing a small artery with destruction of the elastic lamina and media with aneurysm formation. The aneurysm is filled with organizing thrombus.
960
P. D. & LOCKWOOD, C. M , 1987. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis. Lancet, i, 1389-1393. TRAVERS, R. L., ALLISON, D. J., BRETTLE, R. P. & HUGHES,
G. R. V., 1979. Polyarteritis nodosa: clinical and angiographic analysis of 17 cases. Seminars in Arthritis & Rheumatology, 8, 184-198.
The British Journal of Radiology, October 1991
