PULMONARY-HYPERTENSION AS LETHAL COMPLICATION OF EXTRINSIC ALLERGIC ALVEOLITIS

Extrinsic allergic alveolitis is rare in childhood. In addition to the acute form presenting with caracteristic symptoms there is a chronic form with nonspecific symptoms. Our patient presented with severe pulm onary hypertension. After extrinsic allergic alveolitis had been diagn osed,she initially responded well to oral steroid therapy and avoidanc e of antigen exposure. During the following two years she suffered sev eral severe deteriorations as soon as steroid dosage was reduced. Only incomplete elimination of the causative antigen had been achieved. In contrast to the restrictive ventilatory defect the pulmonary hyperten sion remained unchanged by therapy. Discussion: In the treatment of ex trinsic allergic alcveolitis complete elimination of the causative ant igen is the mainstay of therapy. If severe pulmonary hypertension is p resent, treatment should include extended steroid therapy in sufficien tly high doses. With pulmonary hypertension extrinsic allergic alveoli tis should always be taken into account in the differential diagnosis.