Severe Churg–Strauss syndrome with mediastinal lymphadenopathy treated with interferon therapy

doi:10.1016/S0953-6205(02)00136-X

European Journal of Internal Medicine

Volume 13, Issue 7, October 2002, Pages 458-462

https://doi.org/10.1016/S0953-6205(02)00136-X Get rights and content

Abstract

We report a case of severe Churg–Strauss syndrome (CSS) with mediastinal eosinophilic lymphadenopathy, with relapse after standard therapy with steroids and cyclophosphamide, subsequently treated with interferon (IFN) alpha 2b. Our report shows that mediastinal lymph nodes mimicking lymphoma may be one of the clinical manifestations of CSS. We also show that patients with CSS who are resistant to first-line therapy and for whom hypereosinophilia is thought to play an important role may benefit from treatment with IFN.

Introduction

The long-term prognosis for Churg–Strauss syndrome (CSS) is usually good, and cure or remission is obtained in a majority of patients [1]. However, some patients may relapse despite standard therapy; others may require prolonged maintenance treatment with steroids in order to avoid relapses or to control airway obstruction. Treatment may also be interrupted because of side effects. First-line treatment for CSS is prednisolone and cyclophosphamide [2]. When the standard therapy fails to control the disease or is associated with severe side effects, alternative options may include interferon α (IFN-α). We report a case of severe CSS with mediastinal eosinophilic lymphadenopathy that relapsed after a standard regimen but was successfully treated with IFN-α-2b.

Section snippets

Case report

In 1983, a 25-year-old woman developed severe asthma with hypereosinophilia (varying between 800 and 1500 cells/mm³, normal value 0.05–0.8 10³/mm³). She also had a history of nasal polyps with allergic rhinitis and was suspected to have a Widal syndrome. She frequently received steroids for both asthma and allergic rhinitis. In July 1998, the woman presented with pericarditis and was treated with tapering doses of steroids. However, reducing the dose of prednisolone to 20 mg/day precipitated

Discussion

CSS is defined by the combined presence of at least four of the following six criteria [3]: asthma; eosinophilia >10%; history of allergy, mono-, or polyneuropathy; non-fixed pulmonary infiltrates; paranasal sinus abnormality; and extravascular eosinophils. Our patient met all of these criteria. Lymphadenopathy is very rare in CSS. In their cohort of 96 patients with CSS, Guillevin et al. [1] did not mention any patient with lymphadenopathy. Eosinophilic lymphadenopathy has only been described

References (18)

A. Churg et al.
Formes frustes of Churg–Strauss syndrome
Chest
(1995)
P.F. Weller et al.
The idiopathic hypereosinophilic syndrome
Blood
(1994)
D. Aldebert et al.
Eosinophils express a functional receptor for interferon α on the release of mediators
Blood
(1996)
L. Guillevin et al.
Churg–Strauss syndrome. Clinical study and long-term follow-up of 96 patients
Medicine
(1999)
C.A. Langford
Treatment of polyarteritis nodosa, microscopic polyangiitis, and Churg–Strauss syndrome: where do we stand?
Arthritis Rheum
(2001)
A.T. Masi et al.
The American College of Rheumatology 1990 criteria for the classification of the Churg and Strauss syndrome (allergic granulomatosis and angiitis)
Arthritis Rheum
(1990)
T. Kaneki et al.
Churg–Strauss syndrome (allergic granulomatous angiitis) presenting with ileus caused by ischemic ileal ulcer
J Gastroenterol
(1998)
E. Tsuduki et al.
Churg–Strauss syndrome with pulmonary eosinophilia and intrapulmonary lymph nodes 2 years before the onset of vasculitis
Nihon Kokyuki Gakkai Zasshi
(1999)
M. Casey et al.
Lymph node manifestations of limited Churg–Strauss syndrome
J Pediatr Hematol Oncol
(2000)

There are more references available in the full text version of this article.

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Brief reportSevere Churg–Strauss syndrome with mediastinal lymphadenopathy treated with interferon therapy

Abstract

Introduction

Section snippets

Case report

Discussion

Chest

Blood

Blood

Churg–Strauss syndrome. Clinical study and long-term follow-up of 96 patients

Medicine

Treatment of polyarteritis nodosa, microscopic polyangiitis, and Churg–Strauss syndrome: where do we stand?

Arthritis Rheum

The American College of Rheumatology 1990 criteria for the classification of the Churg and Strauss syndrome (allergic granulomatosis and angiitis)

Arthritis Rheum

Churg–Strauss syndrome (allergic granulomatous angiitis) presenting with ileus caused by ischemic ileal ulcer

J Gastroenterol

Churg–Strauss syndrome with pulmonary eosinophilia and intrapulmonary lymph nodes 2 years before the onset of vasculitis

Nihon Kokyuki Gakkai Zasshi

Lymph node manifestations of limited Churg–Strauss syndrome

J Pediatr Hematol Oncol

Brief report
Severe Churg–Strauss syndrome with mediastinal lymphadenopathy treated with interferon therapy