POEMS syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief:

Natural History

• POEMS syndrome typically presents as a chronic progressive polyneuropathy with both sensory and motor disability, the motor component is usually present to a greater degree than the sensory component of the disease.
• Many patients may have coexisting multi-organ system disorders such as organomegaly (liver, spleen and lymph nodes), edema, endocrinopathy (pituitary, thyroid, adrenals), thrombocytosis, polycythemia, pulmonary hypertension, an M-spike (M protein or para-protein) and skin changes. The skin changes include angiomata, hyperpigmentation, hypertrichosis
• The neuropathy is usually symmetrical and ascending, with either insidious or rapidly progressing onset.
• POEMS syndrome may also lead to glomerular disease (membranoproliferative glomerulonephritis) eventually resulting in renal failure.
• If left untreated, patients suffering from POEMS syndrome may become wheel chair bound.

Complications

• Patients suffering from POEMS syndrome may develop the following complications:
  • Renal failure
  • Pulmonary hypertension
  • Pathological fractures (due to lytuc/sclerotic bone lesions)
  • Ischemic stroke
  • Restrictive lung disease
  • Thrombocytosis
  • Polycythemia
  • Myocardial infarction

Prognosis

• The median survival of patients with POEMS syndrome is 165 months.
• Neuropathy, stroke and myocardial infarction are the most common causes of death in POEMS syndrome.