Esophageal involvement as an initial manifestation of Churg-Strauss syndrome

doi:10.1016/j.lpm.2006.10.014

La Presse Médicale

Volume 36, Issue 1, Part 1, January 2007, Pages 57-60

https://doi.org/10.1016/j.lpm.2006.10.014 Get rights and content

Résumé

Introduction

Le syndrome de Churg et Strauss est une vascularite nécrosante et granulomateuse systémique caractérisée par l'asthme, une éosinophilie sanguine et tissulaire et des manifestations extra-pulmonaires.

Observations

Nous rapportons ici 2 observations où elle est révélatrice de la vascularite chez un homme de 56 ans dont la première manifestation clinique était une odynophagie et où elle était associée à des nausées, des vomissements et des douleurs abdominales chez un homme de 35 ans entrant lui aussi dans la maladie par des manifestations digestives. Dans les 2 cas, la tomodensitométrie thoracique montrait un épaississement de l'œsophage initialement et au moment de la rechute.

Discussion

Les atteintes digestives sont fréquentes et une des principales causes de mortalité. L'atteinte oesophagienne est extrêmement rare.

Summary

Introduction

Churg-Strauss syndrome (CSS) is a systemic disease characterized by asthma, blood and tissue eosinophilia, and necrotizing vasculitis with extravascular eosinophilic granulomas.

Cases

We describe two cases of patients with CSS: a 56-year-old man, whose presentation was highly unusual because its initial predominant manifestation was odynophagia, and a 35-year-old man whose disease was diagnosed after a work-up because of nausea, vomiting and abdominal pain. In both cases, thoracic computerized tomography scans showed congestive esophagitis with a markedly thickened esophageal wall at diagnosis and at relapse. Both the vasculitis and the esophageal involvement improved with treatment.

Discussion

Gastrointestinal (GI) involvement is frequent in CSS patients and is one of the major causes of death associated with CSS. Nonetheless, eosinophilic vasculitis-related esophagitis is very rare.

Section snippets

Patient 1

A 56-year-old man, a nonsmoker, had developed asthma approximately 6 weeks before admission for retrosternal pain identified as odynophagia. General examination was normal.

Laboratory findings showed eosinophilia (1800/mm³), but the erythrocyte sedimentation rate (ESR) was normal. Similarly normal were findings from the esophagogastroscopy, colonoscopy, enteroscopy and ileoscopy and the corresponding biopsies. Specifically, the biopsies did not show necrotizing vasculitis. Esophageal manometry

Discussion

Although GI involvement is reported in 8 to 59% of CSS patients 2, 3, it is rarely the first manifestation. A post-mortem study detected GI involvement in 33% of patients [4]. Severe GI involvement represents one of the major causes of death and is an element in the prognostic five-factor score (FFS) we described previously [5]. However, the rarity of esophageal involvement makes it impossible to evaluate its impact on disease severity.

Although vasculitis was histologically proven in only one

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Churg-strauss syndrome
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Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome or rheumatoid arthritis-associated vasculitis
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Gastrointestinal presentation of Churg Strauss syndrome
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Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients
Medicine
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There are more references available in the full text version of this article.

Cited by (11)

Inflammatory bowel disease–like conditions: ischemic bowel diseases and vasculitides
2020, Atlas of Endoscopy Imaging in Inflammatory Bowel Disease
Small and large intestine are common targets for ischemic bowel disease and systemic vasculitides. The presence of edema, erythema, erosions, ulcers, and hemorrhage in those disorders mimics that of inflammatory bowel disease (IBD). In addition, it overlaps in pathogenetic pathways between those disorders and IBD. Tissue ischemia, tissue hypoxia, vasculitis, or vasculopathy may contribute to the etiopathogenesis of IBD. Differential diagnosis is important, as the treatment and prognosis differ. In addition to clinical and radiographic assessment, careful endoscopic characterization of disease distribution and features of inflammation and ulcers and tissue biopsy are valuable for the diagnosis and differential diagnosis.
A 78-year-old woman with an acute eosinophilic gastroenteritis
2011, Clinics and Research in Hepatology and Gastroenterology
Citation Excerpt :
Individualized from periarteritis nodosa in 1951 by Lotte Strauss and Jacob Churg [10], CSS is a rare small vessel-necrotizing vasculitis, ranging from 0.5 to 6.8 cases per million inhabitants a year [11]. Gastrointestinal involvement is quite common (20 to 50%) [11] but usually associated with extra-abdominal signs, reflecting the vasculitis activity [3,8] so that there are few reports of gastroenteritis revealing the disease as in this case [3,12]. CSS’ classical underlining histological abnormalities are parietal fibrinoid necrosis, extravascular necrotizing granulomas and tissue infiltrates by eosinophils [7].
Eosinophil accumulation in the gastrointestinal tract is a common feature of numerous disorders including mainly parasitic infection, drug-induced allergic reactions, inflammatory bowel disease, and various connective tissue disorders. Digestive tissue eosinophilia requires thorough searching for secondary causes that may be specifically treated with antibiotics, dietary and drug elimination or immunosuppressive therapy. Frequency, prognosis and therapeutic implications must guide the diagnostic course. An acute eosinophilic gastroenteritis in a 78-year-old asthmatic woman receiving celecoxib is reported. She presented later with neurologic and cutaneous features and was finally treated by methylprednisolone and cyclophosphamide. The diagnostic approach leading to a Churg-Strauss syndrome (CSS) assertion is described. We discuss the pathogenesis, the management and the potential enhancing role of celecoxib in CSS gastrointestinal involvement.
Esophageal eosinophilia
2010, Surgical Pathology Clinics
Citation Excerpt :
In 2 published cases of Churg-Strauss syndrome presenting in the esophagus, biopsies showed only intense inflammatory cell infiltrates composed mainly of eosinophils. Vasculitis was never identified and the diagnosis was made by applying the American College of Rheumatology classification criteria.60 As in all of the diseases within the differential diagnosis of esophageal eosinophilia, this emphasizes the need to apply clinical context to the findings on histologic examination.
Vasculitides of the gastrointestinal tract
2009, Seminars in Diagnostic Pathology
Citation Excerpt :
General symptoms of CSS include fever, weight loss, and malaise, which are characteristic of multisystem disease. Patients with CSS with GI complaints consisting of abdominal pain, bloody diarrhea, vomiting, ileus, anorexia, bleeding, or odynophagia are nonspecific to CSS.73-75 One report described two cases with congestive esophagitis in patients with CSS after initially presenting with odynophagia.75
Systemic vasculitis is often not considered as a possible diagnosis by clinicians because of its low prevalence compared with other more common diseases. Vasculitis can affect any end organ, and it is therefore often missed early on in disease progression. Gastrointestinal (GI) manifestations of vasculitis are considered rare and the presentation is often nonspecific. However, if there is significant involvement of the major vessels of the gastrointestinal system, life-threatening sequelae, including perforation and bowel ischemia, may occur. This makes early and immediate management crucial to improve long-term morbidity and mortality. Diagnosis of various GI vasculitides often relies on correlation of clinical manifestations with pathology and additional investigations. This paper reviews the various vasculitides that affect the GI tract, including systemic lupus erythematosus, mixed connective tissue disease, Henoch Schönlein purpura, polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, microscopic polyangiitis, enterocolic lymphocytic phlebitis, and Behcet's disease. Segmental arterial mediolysis, mistakenly believed to be a vasculitis, is also discussed.
Eosinophilic esophagitis—established facts and new horizons
2021, Seminars in Immunopathology
Immunoglobulin G4-related disease presenting with clinical similarity to churg-strauss syndrome
2019, Israel Medical Association Journal

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Clinical caseEsophageal involvement as an initial manifestation of Churg-Strauss syndromeUne atteinte œsophagienne. Premier signe clinique d’un syndrome de Churg-Strauss

Résumé

Introduction

Observations

Discussion

Summary

Introduction

Cases

Discussion

Section snippets

Patient 1

Discussion

Allergic granulomatosis, allergic angiitis and periarteritis nodosa

Am. J. Pathol.

Churg-strauss syndrome

Semin. Respir. Crit. Care Med.

Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome or rheumatoid arthritis-associated vasculitis

Medicine

Gastrointestinal presentation of Churg Strauss syndrome

Sarcoidosis

Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients

Medicine

Clinical case
Esophageal involvement as an initial manifestation of Churg-Strauss syndromeUne atteinte œsophagienne. Premier signe clinique d’un syndrome de Churg-Strauss