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Experience of diagnostics and treatment of cardiopulmonary amyloidosis due to familial mediterranean fever (Case study and short review)

https://doi.org/10.28996/1680-4422-2017-2-308-314

Abstract

The article presents a clinical case of amyloidosis cardiopulmonary lesions in a patient with previously established diagnosis of Familial Mediterranean Fever (FMF). In this patient FMF was complicated by renal amyloidosis and end-stage renal disease. Key points of diagnostics were high pulmonary hypertension, echocardiographic feature of restrictive cardiomyopathy and radiological signs of disseminated parenchymal process. The lung damage was interpreted as parenchymal nodular pulmonary amyloidosis after tuberculosis, sarcoidosis, systemic autoimmune diseases and oncology were excluded. The treatment by colchicine was started immediately. Significant positive changes were observed in three months. The signs of disseminated parenchymal process and pulmonary hypertension were completely resolved. The diastolic function of left ventricle also significantly improved. The described case demonstrates the clinical need to examine extrarenal localizations of amyloidosis in patients with FMF and emphasizes the importance of treatment by colchicine even at a late stages of disease.

About the Authors

E. M. Zeltyn-Abramov
Pirogov Russian National Research Medical University (RNRMU); Moscow City Hospital 52
Russian Federation


N. G. Potheshkina
Pirogov Russian National Research Medical University (RNRMU)
Russian Federation


N. I. Belavina
Moscow City Hospital 52
Russian Federation


V. V. Parshin
Moscow City Hospital 52
Russian Federation


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Review

For citations:


Zeltyn-Abramov E.M., Potheshkina N.G., Belavina N.I., Parshin V.V. Experience of diagnostics and treatment of cardiopulmonary amyloidosis due to familial mediterranean fever (Case study and short review). Nephrology and Dialysis. 2017;19(2):308-314. (In Russ.) https://doi.org/10.28996/1680-4422-2017-2-308-314

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ISSN 1680-4422 (Print)
ISSN 2618-9801 (Online)