Summary

Pulmonary amyloidosis is a rare disease that incorporates deposition of amyloid microfibril material in the lung parenchyma. The condition generally presents as an indolent subacute-to-chronic pulmonary disease and requires tissue biopsy to establish the diagnosis. Nodular pulmonary amyloidosis, a subtype of pulmonary amyloidosis, is characterised by special radiographic and pathological features. While the disease can be associated with inflammatory conditions; its association with mucosal-associated lymphoid tissue (MALT lymphoma) is unusual and carries management challenges. Herein, we illustrate a case study of nodular pulmonary amyloidosis associated with underlying MALT lymphoma in a patient with known systemic lupus erythematosus. The aim of this article is to share the management experience of this complex condition with the medical community and to conduct an up-to-date literature review on nodular pulmonary amyloidosis.