Pulmonary hypertension associated with POEMS syndrome

Pulmonary hypertension (PH) is an uncommon but lethal complication of some connective tissue diseases. We describe two cases of PH associated with plasma cell dyscrasia, with polyneuropathy (P), organomegaly (O), endocrinopathy (E), M protein (P) and skin changes (S) described as POEMS syndrome. Two patients, one female and one male, were hospitalized for dyspnoea. Both had a history of POEMS syndrome a few years previously. PH was diagnosed from cardiac catherization, and complete investigations did not reveal other underlying disease. In Case No. 1, mean pulmonary artery pressure was 40 mmHg and pulmonary vascular resistance was 775 dyne x s x cm-5 x m2, with a significant reversibility to vasodilators. Nifedipine and fluindrone treatment was initiated. The patient was still alive 18 months later. In Case No. 2, mean pulmonary artery pressure was 28 mmHg and pulmonary vascular resistance was 243 dyne x s x cm-5x m2, with a slight reversibility to vasodilators. The patient died 6 months later. Our observations suggest that pulmonary hypertension (PH) may be associated with the POEMS syndrome, and that patients suffering from the POEMS syndrome and dyspnoea should be tested for PH.

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