Rheumatology Advance Access published online on September 5, 2008
Rheumatology, doi:10.1093/rheumatology/ken329
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Vasa-vasoritis of the aorta and fatal myocarditis in fulminant Churg–Strauss syndrome
1Internal Medicine Department, Hôtel Dieu and 2Pathology Department, Hôtel Dieu-Immeuble Jean Monnet, Chu Nantes, Nantes, France
Correspondence to:
B. Hervier, Service de Médecine Interne, Hôtel Dieu, Chu Nantes, Place Alexis Ricordeau, 44093 Nantes Cedex 1, France. E-mail: baptiste.hervier{at}chu-nantes.fr
SIR, We report a fulminant case of Churg–Strauss syndrome (CSS) with fatal myocarditis, and atypical aortitis with vasa-vasorum involvement.
CSS is a small-sized vessel necrotizing vasculitis, characterized by late-onset asthma and blood eosinophilia. All organs may be involved, but severe gastrointestinal, renal, cardiac and central nervous system manifestations are associated with a poor prognosis [1].
A 42-yr-old patient was admitted into our institution with a 3-day history of abdominal pain without intestinal bleeding. He was complaining of worsening asthma and nasal polyps. The physical examination was unremarkable, except for sinusal tachycardia.
The ECG was normal, haemoglobin was 13 g/dl, leucocyte count was 16 000/mm3 with 9700 eosinophils/mm3. A parasitological investigation was negative. The serum creatinine was 80 µmol/l, without proteinuria or haematuria. ANCA and muscular biopsy were also negative. A chest X-ray was normal.
On day 3 of hospitalization, the patient died suddenly in ventricular fibrillation. CSS was confirmed at the time of autopsy, revealing a necrotizing myocarditis due to medium-sized vessel vasculitis, with a large eosinophilic infiltrate. There was no evidence of coronaritis. The lungs and liver were also involved, but the intestinal tract and kidneys were normal. Moreover, histological examination of the aorta revealed aortitis with granulomatous and necrotizing angiitis of the vasa-vasorum (Fig. 1).
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Cardiac manifestations are common in CSS and are associated with a poor prognosis [1]. As in our patient, myocardial involvement is a major cause of death [2] and is usually associated with an ANCA-negative status [3].
This case was remarkable because aortitis was noted at the time of autopsy. To our knowledge and in contrast with other ANCA-associated vasculitides [4–9], aortitis has never been reported in association with CSS. Our observation demonstrates that aortitis in CSS is specifically due to involvement of the vasa-vasorum (small-sized vessels), in agreement with the pathogenic hypothesis advanced by Nakabayashi et al. [4] in other ANCA-associated vasculitides. Vasa-vasoritis of the aorta has also been reported in bacterial septicaemia (e.g. Staphylococcus aureus, non-typhi Salmonella spp. and Treponema pallidum) [7, 10]. The lesions we observed were quite different from aortitis occuring in GCA, Takayasu arteritis or the aortitis associated with rheumatic disorders. The lesions appeared closer to Behçet's aortitis involving the media and adventitia.
Clinicians must always be aware of cardiac manifestations of vasculitis, especially in patients with CSS. Aorta can be involved in small-sized vasculitis because of vasa-vasorum involvement.
Disclosure statement: The authors have declared no conflicts of interest.
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