A Rare Case: Primary Pulmonary Amyloidosis

Abstract

Amyloidosis is a rare disorder characterized by the extracellular deposition of insoluble protein aggregates. There are two common types of amyloidosis: Amyloid A (AA) and amyloid light chain (AL). AA amyloidosis typically occurs secondary to chronic inflammatory processes such as tuberculosis and rheumatoid arthritis, with pulmonary involvement being a rare manifestation. AL amyloidosis, on the other hand, is often associated with conditions like multiple myeloma, gammopathies, and idiopathic primary amyloidosis. While kidney involvement is common, pulmonary involvement can also occur as part of systemic amyloidosis or, more rarely, in an isolated form. Pulmonary amyloidosis can present in three forms: nodular, diffuse, and tracheobronchial. This study presented a case of primary pulmonary amyloidosis in the nodular form, identified in a 69-year-old female patient.

Keywords

• Nodular amyloidosis
• amyloid
• light chain
• wedge

Nadir Bir Olgu: Primer Pulmoner Amiloidoz

Abstract

Amiloidoz suda çözünmeyen proteinöz yapıların hücre dışında birikimi ile karakterize nadir görülen bir hastalıktır. Amiloid A (AA) ve amiloid hafif zincir (AL) olmak üzere iki yaygın amiloidoz tipi mevcuttur. AA tipi amiloidoz tüberküloz ve romatoid artrit gibi kronik inflamatuar süreçlere sekonder olarak ortaya çıkar ve pulmoner tutulum nadir bir bulgudur. Öte yandan, AL tipi amiloidoz ise sıklıkla multiple miyelom, gamopatiler ve idiyopatik olarak ortaya çıkan primer amiloidoz gibi durumlarla ilişkilidir. Böbrek tutulumu yaygın olmakla birlikte pulmoner tutulum sistemik amiloidozun bir parçası olarak veya daha nadiren izole bir formda da ortaya çıkabilir. Pulmoner amiloidoz nodüler, diffüz ve trakeobronşiyal olmak üzere üç formda ortaya çıkabilir. Bu çalışmada, 69 yaşında bir kadın hastada saptanan nodüler formdaki primer pulmoner amiloidoz vakası sunulmuştur.

Keywords

• Nodüler amiloidoz
• amiloid
• hafif zincir
• wedge

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