Large bowel obstruction heralding Churg-Strauss syndrome.
- [show abstract] [hide abstract]
ABSTRACT: Churg-Strauss syndrome (CSS) is a systemic vasculitis characterized by the presence of asthma, hypereosinophilia, and necrotizing vasculitis with extravascular eosinophil granulomas. In this retrospective study of 96 patients between 1963 and 1995, we analyzed clinical manifestations, identified prognostic factors, and assessed the long-term outcome. CSS was diagnosed when asthma, hypereosinophilia > 1,500/mm3 or > 10%, and clinical manifestations consistent with systemic vasculitis, with or without histologic evidence, were present. Asthma was the most frequently observed manifestation at presentation, with mononeuritis multiplex the second. Other common manifestations were weight loss, fever, myalgia, skin involvement, paranasal sinusitis, arthralgia, pulmonary infiltrate, and gastrointestinal involvement. Mean eosinophilia at presentation was 7.193 +/- 6.706/mm3; ANCA, present in 20 of 42 (47.6%) patients, predominantly gave the perinuclear labeling pattern. All the patients were treated with corticosteroids alone or in combination with cyclophosphamide or plasma exchanges. Clinical remission was obtained in 91.5%; 22 (25.6%) patients relapsed. Twenty-three patients died during follow-up: 11 of these deaths were directly due to vasculitis. The presence of severe gastrointestinal tract or myocardial involvement was significantly associated with a poor clinical outcome. The long-term prognosis of CSS is good and does not differ from that of polyarteritis nodosa, although most patients need low doses of oral corticosteroids for persistent asthma, even many years after clinical recovery from vasculitis.Medicine 12/1998; 78(1):26-37. · 4.23 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: Six cases of eosinophilic infiltration of the gastrointestinal tract were studied. Three cases were of the diffuse infiltrative variety (eosinophilic enteritis, two cases; eosinophilic peritonitis, one case), and three cases were of the circumscribed variety (so-called inflammatory fibroid polyp). Two of the infiltrative lesions showed necrotizing granulomas identical to those described by Churg and Strauss; one of the two also showed active vasculitis. One circumscribed lesion occurred in a patient with polyarteritis nodosa. Necrotizing eosinophilic granulomas were also noted in this lesion. Our observations suggest that the two forms of eosinophilic infiltration of the gastrointestinal tract are parts of a disease spectrum. Supporting evidence in the literature is presented. The relationship of this group of eosinophilic lesions to the hypereosinophilic syndrome, allergic granulomatosis and angiitis of Churg and Strauss, and polyarteritis nodosa is discussed.Human Pathlogy 02/1979; 10(1):31-43. · 2.84 Impact Factor
- Pathology annual 02/1993; 28 Pt 2:199-220.
American Journal of Gastroenterology
C ?2004 by Am. Coll. of Gastroenterology
Published by Blackwell Publishing
WHAT’S NEW IN GI
Large Bowel Obstruction Heralding
terized by asthma, hypereosinophilia, necrotizing vasculitis,
and extravascular granulomas. Other manifestations include
mononeuritis multiplex, lung infiltrates, skin signs, and gas-
trointestinal (GI) tract involvement (1).
A 60-year-old woman was admitted because of abdom-
inal pain, asthenia, and low-grade fever (37.6◦C). Physical
examination revealed diffuse abdominal tenderness. Labora-
33% eosinophils. The erythrocyte sedimentation rate (ESR)
was 60 mm/Ih and the level of C-reactive protein (CRP)
was 25.9 mg/L (normal <5 mg/L). Total serum IgE were
markedly high (9780 IU/ml, normal range 1–150 IU/ml) and
radioallergosorbent testing revealed the presence of specific
IgE to cat. Antinuclear antibodies were positive (titer 1/160,
“nucleolar” pattern), but anti-dsDNA, anti-extractable
nuclear antigen, and antineutrophil cytoplasmic antibodies
(ANCA) were negative. A chest computer tomography (CT)
was normal, but a plain abdominal X-ray showed a distended
small intestine with air-fluid levels; finally, a barium enema
revealed a stenotic lesion with an “apple core” appearance
involving the ascending colon (Fig. 1A). A laparotomy was
performed, and the terminal ileum and the ascending colon
presence of a 5-cm long circumferential stenotic lesion; mi-
croscopic examination revealed an intense eosinophil-rich
inflammatory infiltrate throughout the intestinal wall but
mainly in the submucosal layer, together with necrotizing
vasculitis of small vessels (Fig. 1B), and eosinophilic gran-
ulomas (Fig. 1C). The mucosa was also involved, with gland
destruction and crypt eosinophilic abscesses (Fig. 1D). A
few days after the operation, multiple skin nodules and ur-
ticarial lesions appeared, and biopsy of one of the nodules
showed leukocytoclastic vasculitis. CSS was diagnosed, and
oral treatment with prednisone and cyclophosphamide was
started, with prompt resolution of the skin lesions and nor-
malization of the eosinophil count, ESR, and CRP. Immuno-
nodules in the left lung and a “ground-glass” appearance of
ment was resumed and 5 months later there were no signs of
in most cases, it affects the stomach and the small bowel,
which are injured as a result of vasculitis of small mesenteric
blood vessels and eosinophilic infiltration of the intestinal
wall (1, 2). The large bowel is rarely involved and most of
the reported cases have features of ischemic colitis (3). The
main clinical feature of our patient at disease onset was large
bowel obstruction, which, to the best of our knowledge, has
not been previously reported.
The histological findings of eosinophilic tissue infiltra-
tion, granulomas, and small-vessel necrotizing vasculitis
were paradigmatic of CSS (1). Eosinophilic infiltration of
the colon is also characteristic of eosinophilic colitis, but
the disease does not usually lead to granulomas or vasculitis
(4, 5). Ulcerative colitis and Crohn’s disease often show tis-
sue eosinophilia and granulomas, but eosinophilic infiltra-
tion is less marked than in CSS and intestinal vasculitis is
rare (4, 5). Polyarteritis nodosa often involves the GI tract
but seldom shows severe tissue eosinophiliaand, as it usually
affects medium-sized vessels, it causes ischemic alterations
in large intestinal segments (6). Wegener’s granulomatosis,
a systemic necrotizing vasculitis, sometimes presents with
marked eosinophilia and can thus mimic CSS; in our patient,
both the absence of ANCA and the clinical history of allergy
made CSS a more likely diagnosis.
although CSS is usually considered a systemic disease, some
patients present with “limited forms of CSS” (eosinophilic
vasculitis and/or extravascular granulomas in isolated organs
or tissues). The diagnosis of limited CSS does not preclude
the possibility of a disease continuum: such as it occurs in
also CSS may in time progress from a limited to a dissem-
inated disease (8). In our patient CSS initially only affected
the large bowel, but subsequently also involved the skin and
The present case extends the clinical spectrum of CSS to
large bowel obstruction, and also highlights the possibility
that, although CSS may be limited to a single organ at onset,
it can subsequently become systemic.
Augusto Vaglio, M.D.
Domenico Corradi, M.D.
Nicoletta Ronda, M.D., Ph.D.
Giovanni Garini, M.D.
Carlo Buzio, M.D.
Department of Clinical Medicine,
Nephrology and Health Science,
and Department of Pathology and Laboratory Medicine,
University of Parma, Parma, Italy
B. Vasculitis of a small artery of the colonic submucose, with eosinophilic infiltrate in and around the blood vessel wall, and a peripheral
area of neorosis (arrows). Bar: 100 µm. Hematoxylin-eosin, original magnification × 10. C. Eosinophil-rich granulomatous inflammation
mucosae. Bar: 100 µm. Hematoxylin-eosin, original magnification × 10. D. Diffuse eosinophil-rich inflammatory infiltrate of the colonic
mucosa with crypt abscesses (arrows). Bar: 50 µm. Hematoxylin-eosin, original magnification × 20.
1. Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syn-
Medicine (Baltimore) 1999;78:26–37.
the gastrointestinal tract and liver. Gastroenterol Clin North
3. Shimamoto C, Hirata I, Ohshiba S, et al. Churg-Strauss syn-
drome (allergic granulomatous angiitis) with peculiar multi-
ple colonic ulcers. Am J Gastroenterol 1990;85:316–9.
4. Nonneoplastic lesions of the colon. In: Fenoglio-Preiser CM,
Noffsinger AE, Stemmermann GN, et al., eds. Gastrointesti-
nal pathology. 2nd edition. Philadelphia: Lippincott-Raven
6. Suen KC, Burton JD. The spectrum of eosinophilic infil-
tration of the gastrointestinal tract and its relationship to
other disorders of angiitis and granulomatosis. Hum Pathol
7. Masi AT, Hunder GG, Lie JT, et al. The American Col-
lege of Rheumatology 1990 criteria for the classification of
Churg-Strauss syndrome (allergic granulomatosis and angi-
itis). Arthritis Rheum 1990;33:1094–100.
8. Lie JT. Limited forms of Churg-Strauss syndrome. Pathol
Annu 1993;28(Pt 2):199–220.
timento di Clinica Medica, Nefrologia e Scienze della Prevenzione.
Universit` a degli Studi di Parma, Via Gramsci 14, 43100, Parma,
Received June 20, 2003; accepted October 27, 2003.