[POEMS syndrome with chronic renal failure]. / POEMS-Syndrom mit Niereninsuffizienz.

ABSTRACT

CASE HISTORY AND CLINICAL FINDINGS: A 43-year-old male was admitted to the hospital because of subfebrile temperatures since 3 months, weight loss of 9 kilogramms and lateral foot pains with bilateral leg edema. Over the last years, the patient had consulted a doctor several times for upper respiratory infections, orchitis and a spinal neck syndrome. At the physical examination, we found a temperature of 37.4 degrees C, no rales, no crackles at auscultation of the lung, bilateral ankle edema and livid, pressure-dolent skin changes at the lateral margins of both feet. The blood pressure was 170/100 mmHg. EXAMINATIONS The following pathologically laboratory results were found erythrocyte sedimentation rale (ESR) 35/55 mm n. W., C-reactive protein (CRP) up to 39 mg/l, leucocytes up to 13.5/nl, LH and FHS were elevated corresponding to hypergonadotropic hypogonadism, renal failure with at the beginning a selective glomerular proteinuria, as well as a monoclonal IgG-gammopathy. The bone marrow aspiration as well as the bone marrow biopsy revealed neither plasmocytoma nor a malignant systemic disease. The ultrasound examination showed enlarged liver, spleen, and kidneys. TREATMENT AND FOLLOW-UP After excluding a connective tissue disease and an infection and with the missing proof of a malignant tumor treatment was started with parenteral methylprednisolon 500 mg on 3 consecutive days under the hypothesis of classic panarteriitis nodosa, even when multiple biopsies were negative. Under the treatment, the elevated inflammatory parameters and renal failure improved, but deteriorated quickly after discontinuation of the corticosteroid medication. The second kidney biopsy showed a chronic scaring glomerulopathy of the hemolytic uremic syndrome type. In the follow-up the renal insufficiency was improved by corticosteroids. With the manifestation of a bilateral sensory polyneuropathy in January 1994, the diagnosis of a POEMS syndrome was most likely. CONCLUSION: According to the literature, up to 50% of the cases with POEMS syndrome reveal renal failure. Most times a glomerular microangiopathy is shown histologically. A treatment trial with corticosteroids is justified.