02 October 2013 21 1K Report

A 15 year old girl presented with exertional SOB for last 1 month which exacerbated for last 2 days. At OPD she was grade 4 Dyspneic with resting desaturation with cyanosis and following cxr. the patient had repeated sputum for AFB which were found to be negative. ELISA for HIV 1&2 was negative. the patient had dramatic response in improvement of SOB with pulsed methylprednisolone but deteriorated again after discontinuation. ABG was suggestive of PaO2/FiO2 less than 200 with increased D(A-a)O2. Patient partly denied mechanical ventilatory support.. as long as pt was on IV methylpred she remained stable but deteriorated just within 24 hr of its discontinuation. TLC normal, CRP raised, serum ANA negative, serum RA factor slightly raised. What may be the possibilities?

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