Large bowel obstruction heralding Churg-Strauss syndrome.
- SourceAvailable from: Augusto Vaglio[Show abstract] [Hide abstract]
ABSTRACT: To explore the association between HLA alleles and Churg-Strauss syndrome (CSS), and to investigate the potential influence of HLA alleles on the clinical spectrum of the disease. Low-resolution genotyping of HLA-A, HLA-B, and HLA-DR loci and genotyping of TNFA -238A/G and TNFA -308A/G single-nucleotide polymorphisms were performed in 48 consecutive CSS patients and 350 healthy controls. The frequency of the HLA-DRB1*07 allele was higher in the CSS patients than in controls (27.1% versus 13.3%; chi(2) = 12.64, P = 0.0003, corrected P [P(corr)] = 0.0042, odds ratio [OR] 2.42, 95% confidence interval [95% CI] 1.47-3.99). The HLA-DRB4 gene, present in subjects carrying either HLA-DRB1*04, HLA-DRB1*07, or HLA-DRB1*09 alleles, was also far more frequent in patients than in controls (38.5% versus 20.1%; chi(2) = 16.46, P = 0.000058, P(corr) = 0.000232, OR 2.49, 95% CI 1.58-3.09). Conversely, the frequency of the HLA-DRB3 gene was lower in patients than in controls (35.4% versus 50.4%; chi(2) = 7.62, P = 0.0057, P(corr) = 0.0228, OR 0.54, 95% CI 0.35-0.84). CSS has 2 major clinical subsets, antineutrophil cytoplasmic antibody (ANCA)-positive, with features of small-vessel vasculitis, and ANCA-negative, in which organ damage is mainly mediated by tissue eosinophilic infiltration; analysis of HLA-DRB4 in patients categorized by different numbers of vasculitic manifestations (purpura, alveolar hemorrhage, mononeuritis multiplex, rapidly progressive glomerulonephritis, and constitutional symptoms) showed that its frequency strongly correlated with the number of vasculitis symptoms (P for trend = 0.001). These findings indicate that HLA-DRB4 is a genetic risk factor for the development of CSS and increases the likelihood of development of vasculitic manifestations of the disease.Arthritis & Rheumatology 10/2007; 56(9):3159-66. · 7.48 Impact Factor
Article: Churg-Strauss syndrome.[Show abstract] [Hide abstract]
ABSTRACT: Churg-Strauss syndrome is a rare diffuse vasculitis that is almost invariably accompanied by severe asthma. Although overall prognosis is good, and treatment with prednisone alone or in combination with immunosuppressive drugs is usually successful, severe asthma typically persists. Diffuse organ involvement of Churg-Strauss syndrome, especially cardiovascular and rare involvement of the CNS and renal system, suggests a poorer prognosis than usual, and can be fatal. The cause of Churg-Strauss syndrome is unknown, but its characteristic histological findings and association with asthma distinguish it from other vasculitides. Controversy surrounds the use of asthma drugs-especially antileukotrienes--and development of the disorder. We review the epidemiological evidence for an association of drug treatment with Churg-Strauss syndrome, the diverse diagnostic and pathological criteria for this syndrome, and treatment options.The Lancet 03/2003; 361(9357):587-94. · 39.21 Impact Factor
Article: Churg-Strauss syndrome.[Show abstract] [Hide abstract]
ABSTRACT: ANCA, asthma, Churg–Strauss syndrome, eosinophils, HLA, vasculitisKidney International 07/2009; 76(9):1006-11. · 8.52 Impact Factor
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.
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drome (allergic granulomatous angiitis) with peculiar multi-
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4. Nonneoplastic lesions of the colon. In: Fenoglio-Preiser CM,
Noffsinger AE, Stemmermann GN, et al., eds. Gastrointesti-
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lege of Rheumatology 1990 criteria for the classification of
Churg-Strauss syndrome (allergic granulomatosis and angi-
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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.