Primary pulmonary systemic amyloidosis - case report

Abstract

Pulmonary amyloidosis is a rare disease, characterized by extracellular deposition of fibrillary protein in the lungs. Primary pulmonary amyloidosis is rare disease, that present focal or systemic characteristics. The secondary system form is generally related to neoplastic, infectious, or chronic inflammatory processes, chronic kidney disease, syphilis, leprosy, inflammatory bowel disease, osteomyelitis, parasitic infections, rheumatoid arthritis. We present the case of patient with diffuse pulmonary amyloidosis Key words: pulmonary amyloidosis, pathology myloidosis is not a single disease but a term for diseases that share a common feature: the extracellular deposition of pathologic insoluble fibrillar proteins in organs and tissues. In the mid-19 th century, Virchow adopted the botanical term "amyloid", meaning starch or cellulose, to describe abnormal extracellular material seen in the liver at autopsy [36]. Subsequently, amyloid was found to stain with Congo red, appearing red microscopically in normal light but apple green when viewed in polarized light [1,8]. In major development was the recognition that amyloid fibrils in primary amyloidosis are fragments of immunoglobin light chains [15]. Subsequently, it was determined that different proteins made up the amyloid fibrils in reactive [secondary] amyloidosis and familial amyloidosis [2], opening the way to specific therapies designed to target the source of fibrilprecursor production [9]. Amyloidosis is characterized by deposition of abnormal protein, fibrillary proteins material in extracellular tissue, in a variety of organs [9, 13, 31, 32]. It is a generic term for a heterogeneous group of diseases, including Alzheimer's disease and type II diabetes mellitus [28]. It can be hereditary or acquired potentially fatal or a merely accidental finding [13]. In a simplified way, the disease can be subdivided into localized or systemic form. It can also be classified as primary or secondary [23]. The respiratory system is frequently involved in patients with amyloidosis [6, 9, 17, 21, 35]. Amyloidosis of the respiratory tract was first described in 1877 by Lesser [31].

A rare case of multinodular pulmonary amyloidosis

“…According to immunohistochemistry, there are four major categories of amyloidosis: (i) primary or immunoglobulin light chain (AL) disease; (ii) secondary or amyloid protein A disease; (iii) hereditary or mutant transthyretin disease; and (iv) dialysis‐associated or β 2 ‐microglobulin disease. The AL fibril is a common type of amyloid deposit in the lung, usually the deposit is systemic . However as in our case, the AL type was confirmed without systemic deposit.…”

“…Firstly, the disease is often undiagnosed or misdiagnosed especially in Asian countries in the past because doctors had insufficient understanding of pulmonary amyloidosis. Secondly, selection bias potentially makes data from tertiary referral centers unrepresentative .…”