PULMONARY-HYPERTENSION IN POEMS SYNDROME - A NEW FEATURE MEDIATED BY CYTOKINES

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammop athy, and skin changes) syndrome is a rare variant of plasma cell dysc rasia with multiple systemic manifestations. We followed the progress of 20 patients with POEMS syndrome in our institution over a 10-yr per iod. Pulmonary hypertension (PH) was observed in five patients. All pa tients suffered dyspnea on exertion, which always appeared during an e xacerbation of POEMS syndrome. The typical echocardiographic signs of PH were observed in all of these patients, and the median pulmonary-ar tery systolic pressure was 57 mm Hg (range, 50 to 65 mm Hg). Mean pulm onary-artery pressure during right side heart catheterization in two p atients was 32 mm Hg. No other explanation for the PH could be found. Overproduction of cytokines was found in all cases, with high serum co ncentrations of interleukin-1 beta, interleukin-6, tumor necrosis fact or-alpha, and vascular endothelial growth factor. We suggest that PH s hould be added to the list of symptoms of POEMS syndrome. Cytokines ma y mediate POEMS syndrome-associated PH, as proposed for the other syst emic manifestations of this disorder.