Reversal of pulmonary arterial hypertension in POEMS syndrome with thalidomide: a case report

Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease characterized by the remodelling of distal pulmonary arteries in the absence of other cardiopulmonary disease, usually leading to right ventricular failure. Given the current European Society of Cardiology and the European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension (PH), most of the patients with severe PAH usually require to have a lifelong combination therapy that includes prostacyclin, phosphodiesterase-5 inhibitor, and endothelin receptor antagonist. However, the reversibility of PAH has been reported through the treatment of the underlying diseases or comorbidities.

We present a case of a 45-year-old woman with a chief complaint of dyspnoea, eventually diagnosed with severe PAH in the setting of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome that was successfully treated with thalidomide and dexamethasone.

Our case suggests that it would be important to consider associated syndromes when a diagnosis of PH is made, because treatment of the underlying condition may lead to improvement in PAH.