Churg Strauss Syndrome – A Case Report

in two three doses, once in three weeks x three doses. Followed by maintenance with Azathioprine oral Prednisolone in tapering doses. And other medication with aspirin, statins, ACE inhibitors, isosorbide physiotherapy. and aBstRaCt A male aged 45-years presented with complaints of fever for 7days, cough, breathlessness for 4 days, tingling of hands, feet and weakness of both hands and feet for 4 days duration. He was a known asthmatic with history of recurrent sinusitis in the past. On examination he had bilateral polyphonic wheeze and evidence of distal asymmetric sensory neuropathy with motor weakness. And on investigation he had eosinophilia, pulmonary function test showed reversible airway obstruction,nerve conduction studies revealed mononeuritis multiplex, muscle biopsy suggestive of eosinophilia infiltration, nerve biopsy suggestive of vacuities, 2DECHO showed RWMA in inter ventricular septum, Coronary angiogram showed narrowing in distal LAD territory. In view of sinusitis, asthma, eosinophilia, tissue infiltration by eosinophils we made a diagnosis of Churg Strauss syndrome.

His general examination was unremarkable. Pulse rate was 108/ minutes, respiratory rate 28/minutes and blood pressure was 120/70 mm Hg. Respiratory system examination revealed bilateral expiratory Ronchi. Nervous system examination revealed bilateral asymmetrical distal muscle weakness with sensory loss. Deep Tendon Reflexes were not elicitable in both upper and lower limbs, except the Biceps reflexes. Other system examination were normal.
Investigations revealed hemoglobin 11.7 gm/dl, total Leukocyte count 21,700 cells/mm 3 , (Neutrophils 64%, Lymphocytes 15%, Monocytes 1% and Eosinophils 20%) [Table/ Fig-1], platelet count 3.0 lakhs/mm 3 , ESR 70 mm and absolute eosinophil count was 4000cells/mm 3 . Renal function tests were normal but had mild Hepatopathy. Creatinine phosphokinase was 532U/L (normal upto 200 U/L) CPK-MB is normal, Troponin T-test was negative. There was sinus tachycardia in Electrocardiogram but Echocardiogram showed hypokinesia in septum and anterior wall. Coronary angiogram showed mild single vessel disease in Left anterior descending coronary artery territory. Chest radiograph was normal. There were no ova and cysts in stools. Smear for microfilaria was negative. ANA, anti dsDNA and c-ANCA were negative. P-ANCA (perinuclear antineutrophil cytoplasmic antibody) (ELISA) was positive.
Nerve conduction Studies were suggestive of mononeuritis multiplex. There was reversible airway obstructive abnormality on spirometry. His base line FEV1 was 68% of predicted value witch increased to 90% of predicted FEV1 after bronchodilator testing suggestive of reversible airway obstruction. Bone marrow study revealed increased number of eosinophils and their precursors. There was no blast prominence [  -3]: Nerve biopsy shows patchy demyelination of Neurons symmetrical or asymmetrical polyneuropathy [5]. In our case it was Fulminant form of mononeuritis multiplex. Muscle biopsy helps in the demonstration of systemic vasculitis [6], as in our case insert as muscle biopsy also proven that the presence of extravasular eosinophils and it was one of the criteria for diagnosis of CSS.
Corticosteroids are the first-line recommended treatment for CSS and adjuvant immunosuppressants are used for severe disease [7,8]. Our patient was considered to have severe disease and he was thus treated with combination therapy immediately Intravenous cyclophosphamide was preferred as it has been shown to cause fewer adverse effects than oral cyclophosphamide. Corticosteroids and immunosuppressants, especially cyclophosphamide, have considerably improved the prognoses and overall survival rates in patients with systemic vasculitis.

ConClusion
We conclude that, Churg-Strauss syndrome complicated frequently with Fulminant form of polyneuropathy, and Cardiac involvement. Remission depends on early aggressive immunosuppressive therapy and long term follow-up. Overall the prognosis of a treated case of CSS appears good with adequate treatment with immunosuppressive medication.  [2]. Regularly, a history of asthma and rhinosinusitis precedes the appearance of the syndrome itself [3]. In our case asthma was present years before the CSS. Cardiac involvement occurs frequently in CSS, and results in significant morbidity and mortality [4]. Cardiac involvement may result from vasculitis-related ischemia affecting small myocardial vessels and coronary arteries, and from eosinophilic myocardial infiltration. Our case also had regional wall motion abnormality in 2D echocardiography but Coronary angiogram did not reveal any significant disease this patient may had vasulitis involving small myocardial blood vessels. The pattern of neurological involvement may be that of mononeuritis multiplex,