INTRODUCTION:Churg strauss syndrome (CSS) is a vasculitic syndrome consisting of asthma, peripheral eosinophilia and necrotizing granulomas. We report a novel case of CSS with tracheal and endobronchial involvement without significant parenchymal lung involvement.
CASE PRESENTATION:A 58 year old white male with a five year history of asthma presented with ten days of progressive paresthesias and weakness. He initially noted tingling and numbness starting in his left foot and spreading to other extremities. He developed weakness of his left hand and right foot, later involving all other extremities. He also had a rash on the dorsa of both hands. He also had progressively worsening dyspnea and wheezing over several months. He had cough with minimal sputum production and no hemoptysis. He did not have fevers, chills, rigors, or weight loss. His past medical history is significant for asthma, hypertension, and GERD. He is a retired factory (administrative) worker. He lives with wife, has no pets or recent travels. He is a lifelong nonsmoker and social drinker. Medications include prednisone 10 mg (started for asthma exacerbation and being tapered) fluticasone/salmeterol, aspirin, celecoxib, esomeperazole, levalbuterol and montelukast since one year. He has recently required intermittent steroid courses or asthma exacerbations.On examination, he was afebrile and vital signs were stable. He was requiring 0.28 FiO2 to keep saturation of 98%. His lungs had bilateral scattered wheezes. He had multiple punctate erythematous lesions involving both hands dorsally. His left hand was diffusely numb. He had decreased sensations in right hand and both feet upto his knees. Strength was decreased in all distal muscles and he had difficulty standing and walking. WBC count was 11K/μL with a normal differential. His metabolic profile was within normal limits.ANA was negative, rheumatoid factor was 46IU/ml. C-ANCA was positive (1:20) with negative P-ANCA. Myeloperoxidase antibody was positive. IgE was 1140 IU/m. Lumbar puncture and cardiac echo were normal. Evaluation of the upper airway by nasopharyngoscopy revealed no abnormalities other than changes of GERD. A HRCT showed no pulmonary infiltrates. Pulmonary function tests showed moderate obstruction with normal diffusion capacity. Electromyography showed asymmetric sensori-motor polyneuropathy. A skin biopsy and nerve biopsy were nondiagnostic. The montelukast was stopped and a five day course of high dose steroids pending biopsy results improved his symptoms. Off steroids, he felt more dyspneic and hypoxemic with recurrence of his neuropathy. Repeat CBC now showed peripheral eosinophilia (19%). Bronchoscopy revealed diffuse tracheal and endobrochial mucosal lesions. Biopsy of these lesions was consistent with allergic angiitis and granulomatosis (Churg-Strauss syndrome). He was started on prednisone and cyclophosphamide with significant improvement in dyspnea and hypoxemia.
DISCUSSIONS:Tracheobronchial lesions in CSS are rare, with only three cases reported in the literature. More than 90% of cases with CSS have parenchymal abnormalities on HRCTs, as did these three previous cases. We have not found any previously reported cases of CSS with only tracheobronchial involvement. This case of CSS with tracheobronchial involvement is unique in that no pulmonary infiltrates were seen on HRCT and diffusion capacity was normal.
CONCLUSION:CSS is a syndrome that can present very indolently with asthma-like symptoms and is often masked by concomitant systemic steroids. The diagnosis of CSS should be considered in any patient with worsening asthma and symptoms suggestive of vasculitis even in the absence of any radiologic infiltrates.
DISCLOSURE:Kiran Devulapally, No Financial Disclosure Information; No Product/Research Disclosure Information