Lymphocytic interstitial pneumonia and pulmonary embolism in a patient with tetralogy of Fallot and common variable immunodeficiency: is there any link?

Abstract

We report the simultaneous occurrence of lymphocytic interstitial pneumonitis and pulmonary embolism in a patient with tetralogy of Fallot and common variable immunodeficiency. An explanation based on the pathogenesis of these diseases is proposed to explain this ''unfortunate coincidence''.A 45-year-old Caucasian woman with tetralogy of Fallot, surgically corrected at age 6 years, presented with severe dyspnoea and an acute episode of pleuritic right-sided chest pain, fevers of 102uF and chills. She was empirically treated with antibiotics and steroids for pneumonia twice in the previous 12 weeks, without improvement in her dyspnoea. She had a 20 pack-year smoking history but denied the use of illegal drugs or alcohol.On admission she was afebrile, haemodynamically stable with an oxygen saturation of 78% on room air. The lungs exhibited diffuse expiratory ronchi and wheezes. A grade II/VI systolic ejection murmur and an early diastolic murmur were noted at the upper left sternal border. Extremities revealed distal cyanosis, but no clubbing or oedema. White blood cell count was 20 000/mm 3 with 96% neutrophils and 3% lymphocytes and b-natriuretic peptide level was 256 pg/ml.