Description of rare clinical case of AA-amyloidosis with involvement in pathological process of heart and lungs

Abstract

Amyloidosis is a unique group of disorders caused by the insoluble protein fibers accumulation known as amyloid fibrils in extracellular tissue and organ spaces. The involvement of different organs and tissues in amyloidosis is often the cause of missed or delayed diagnosis, and amyloidosis remains a major clinical problem as it is associated with 1/1,000 deaths in developed countries. We present a case of a 59-year-old female patient with complaints of breath shortness while talking, a sense of air lack in the horizontal position, swelling of the lower limbs, reduction of blood pressure to a minimum of 70/50 mm Hg, weight reduction by 7 kg in 6 months, dizziness. The patient was hospitalized to the Cardiology Department of Scientific Research Institute – Ochapovsky Regional Clinical Hospital #1 with heart failure. The electrocardiogram revealed left ventricular hypertrophy. Echocardiography showed biventricular myocardial hypertrophy with a maximum interventricular septum thickness of 16 mm, nonuniform ventricular myocardial structure and reduction of global left ventricular comparability to 38%. Computer tomography of chest organs demostrated interstitial swelling of parenchyma of both lungs. The infection during the further examination. For the final morphological verification of the diagnosis, it was necessary to perform peripheral transbronchial biopsy S 3, 4, 5 of the left lung and myocardial biopsy with specific staining on amyloid-congo – red. In the lung and myocardial biopsy, the colour of the congo red is positive. An immunohistochemical study of the lung tissue and myocardial sample revealed Amyloid AA expression. This case represents a rare cause of heart failure in a woman. A feature of this observation is the rare involvement in the pathological process of both pulmonary tissue and myocardium with AA-amyloid deposition.