Systemic corticosteroids as first-line treatment in pulmonary hypertension associated with POEMS syndrome.

The POEMS syndrome is a rare plasma cell disease. Pulmonary hypertension is an infrequent respiratory complication of this syndrome and might be associated with increased levels of various cytokines, chemokines and growth factors as part of the inflammatory phenomena that involve the physiopathology of POEMS syndrome. We present the case of a 54-year-old woman diagnosed with POEMS syndrome and pulmonary hypertension, which were treated with corticosteroids as the first-line therapy. The patient presented with the classic symptoms of this syndrome: polyneuropathy (confirmed by electromyography), organomegaly, subclinical hypothyroidism and monoclonal gammopathy detected in urine, together with skin changes. Right heart catheterization revealed a mean pulmonary artery pressure of 48 mmHg, a cardiac output of 4.1 L/min and pulmonary vascular resistance of 8.05 Woods. The serum level of brain natriuretic peptide (BNP) was 150 pg/mL. No other underlying disease was found during the investigation. Prednisone (1 mg/kg for three months) was then initiated, with a dramatic improvement in the clinical and functional condition. Levels of thyroid hormones and urinary protein levels (as determined using electrophoresis) normalized. Mean pulmonary artery pressure decreased to 26 mmHg, cardiac output decreased to 3.8 L/min, and pulmonary vascular resistance decreased to 2.89 Woods. Serum levels of BNP dropped to 8 pg/mL. Our findings suggest that corticosteroids could play a role as a first-line treatment in pulmonary hypertension accompanied by POEMS syndrome. Due to the rarity of this presentation, a multicenter registry should be developed to allow the compilation of additional data to support this practice.