Department of Internal Medicine II and 1Department of Dermatology, University of Kiel, Chemnitzstrasse 33, D-24116 Kiel, Germany
Correspondence to:
C. Beck. E-mail: c.beck{at}med2.uni-kiel.de
SIR, We present here the case of a patient experiencing persistent aphonia months prior to the diagnosis of ChurgStrauss syndrome (CSS). The patient gave informed consent and the report conforms to standards currently applied in Germany.
A 57-yr-old female patient reported a loss of smell starting a year before admission, followed by asthmatic attacks months later. Hoarseness and subsequent complete loss of vocalization led to examination by two otolaryngologists and functional dysphonia and chronic laryngitis were diagnosed respectively. Antibiotic treatment did not improve symptoms. Two months later she was seen again owing to inflammation of the left outer auditory canal. She later complained about numbness in her fingertips and soles. Exacerbation of her asthmatic attacks and rapidly progressive vasculitis finally led to hospitalization. Her past medical history included chronic sinusitis and coeliac disease, which was diagnosed 10 yr earlier.
On admission we saw a 57-yr-old white, non-obese female (170 cm, 59 kg) with typical symptoms of bronchial asthma. A slight systolic murmur was heard. Pulse, blood pressure and axillary temperature were normal. On both lower legs extensive skin necrosis and localized oedema were seen. Pulses of foot arteries were not palpable. She had a white blood cell count of 33 000/µl with 64% eosinophils, IgE was 1310 mg/l, anticytoplasmic antibody (ANCA) titre was 1:160 and C-reactive protein 135 mg/dl. A skin biopsy revealed a leucocytoclastic vasculitis. ECG and echocardiogram were normal, a chest radiograph did not show pulmonary abnormalities, an MRI scan of the skull revealed a polyp in the left maxillary sinus and signs of chronic mastoiditis. Palsy of the recurrent laryngeal nerve and subglottic stenosis were not diagnosed by otolaryngologists.
The patient was treated with i.v. methylprednisolone starting with 250 mg for 3 days and then gradually tapered. A more aggressive treatment was not considered as no other life-threatening organ involvement was present. In addition she received inhalatory corticosteroids for treatment of her bronchial asthma and 200 mg of carbamazepine three times a day for treatment of lancinating pain in her lower legs (interpreted as multiplex polyneuritis). Pantoprazole (40 mg) was given as prophylaxis against gastric ulcers. Ibuprofen was gradually reduced and the patient did not take any pain medication on discharge after 14 days.
The necrotizing vasculitis responded well to treatment, her voice regained strength, and white blood cell and differential blood count returned to normal. Spirometry performed the day before discharge still showed moderate obstruction.
A striking feature in the clinical course of CSS presented here, the acute onset of aphonia, is hardly ever reported [1]. Nasal and sinusoidal disorders are common in Wegener's granulomatosis, CSS and sarcoidosis [2], crusting rhinitis and chronic sinusitis being the most frequent manifestations [3]. Neurogenic damage (revealed by laryngeal electromyography of the thyroarytenoid and cricoarytenoid muscles) has been reported as a sign of CSS [1] and may cause hoarseness or aphonia, but paresis of the vocal cord was not diagnosed here. Subglottic stenosis in association with the presence of antineutrophil antibodies [4] has been reported, but was excluded here.
The presence of five diagnostic ACR-classification criteria (asthma, eosinophilia, mononeuritis, affection of the paranasal sinuses and eosinophil extravasation) allows the diagnosis of CSS with a specificity of 99% and sensitivity of 85% [5] (http://www.rheumatology.org/;). The diagnosis is further supported by detection of ANCA [6]. Lack of pulses below the knee and extensive necrosis of the skin suggested the involvement of larger arteries, but angiography was declined by the patient. Weight loss, fever and arthralgia, cardiac and renal involvement were absent, so standard immunosuppressive therapy was given [7].
Another remarkable feature of the presented case is the fact that coeliac disease preceded the onset of CSS. The patient had been on a gluten-free diet ever since diagnosis. Initial symptoms of the prodromal phase of CSS had an onset 10 yr later and no association between CSS and coeliac disease has been reported, although there is a series of reports linking coeliac disease to angiitis and Behçet's disease [810]. Due to the very long latency period between the initial diagnosis of coeliac disease and vasculitis, a direct link between the two cannot be proved in our case. Whether patients with coeliac disease may have a predisposition for vasculitis in general remains speculative.
We report here the very rare occasion in which complete aphonia preceded CSS in a very interesting constellation of coeliac disease preceding this complex symptomatology.
The authors have declared no conflicts of interest.
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