Pulmonary Amyloidosis – Keeping an Eye Open to the Uncommon: A Case Series
DOI:
https://doi.org/10.21276/apalm.3930Keywords:
pulmonary amyloidosis, AL amyloidosis, Congo red staining, apple-green birefringence, fibrillary protein deposition, nodular pulmonary amyloidosisAbstract
Introduction: Pulmonary amyloidosis is a disorder characterized by extracellular deposition of misfolded protein fibrils that disrupt organ function. It is rare and frequently underrecognized due to its clinical and radiological overlap with other pulmonary disorders including interstitial lung disease and malignancy.
Case Presentation: Here, we have described seven cases of pulmonary amyloidosis that we encountered over a period of 7 years in our routine lung pathology practice. The patients were aged 45–79 years with a female predominance and showed diffuse alveolar-septal, nodular, and tracheobronchial patterns of involvement. Five patients had multisystem AL amyloidosis with concurrent cardiac, renal and/or gastrointestinal involvement, while two cases showed tracheobronchial and nodular pulmonary amyloidosis without systemic involvement. Diagnosis was established by Congo red positive deposits on biopsy with apple green birefringence on polarized microscopy and supported by further evaluation including amyloid typing in a few. Management varied according to the disease extent, with plasma cell directed therapy for systemic disease and conservative or site-directed treatment for localized forms.
Conclusion: Pulmonary amyloidosis is a rare and heterogeneous condition and its mimicry of other commoner diseases often causes diagnostic delay. Accurate diagnosis requires a high index of suspicion both clinically and on histopathological evaluation with inclusion of ancillary tests like Congo-red staining, and further subtyping of amyloid protein for efficient management.
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Copyright (c) 2026 Ruby Fathim S Ellikkal, Bhavika Sharma, Jyotsna Yesodharan
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