A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis

Abstract

Amyloidosis is a heterogeneous group of disorder associated with the deposition of protein in an abnormal fibrillar form. Primary Sjögren's syndrome (PSS) is a systemic inflammatory disorder that commonly affects the exocrine glands. The reported frequency of pulmonary involvement in PSS varies widely, ranging from 9% to 75%. Pulmonary involvement occurs in light-chain (AL) amyloidosis and is uncommon in the reactive (AA) and hereditary forms. Herein we present a case of PSS associated diffuse multinodular amyloidosis in the lung. We followed up the patient without treatment for three years. There are only minimal lung symptoms related to lung infiltration. In conclusion, pulmonary involvement in SS is an extremely rare clinical manifestation and usually has a good survival rate without treatment.

18F-FDG PET/CT in Patients with Nodular Pulmonary Amyloidosis: Case Report and Literature Review

“…Pulmonary involvement rarely causes symptoms unless gas exchange in alveolar structures is severely affected by amyloid deposits [ 2 , 18 , 24 ]. Histologically, amyloid deposits are identified on the basis of eosinophilic amorphous deposits which take up Congo red stain and typically exhibit apple-green birefringence when examined under polarized light [ 1 , 7 , 22 , 24 , 25 ].…”

“…The size of the nodules varies from a few millimeters to several centimeters. It is usually a silent disease and found incidentally on chest radiographs in asymptomatic, older individuals [ 17 , 18 , 19 , 24 , 25 ]. It may show a slow progression of increased size or number of nodules but not always reveals a restrictive pattern of lung function or impairment of gas exchange.…”

“…It may show a slow progression of increased size or number of nodules but not always reveals a restrictive pattern of lung function or impairment of gas exchange. The natural history of nodular pulmonary amyloidosis is associated with a relatively benign prognosis [ 2 , 18 , 19 , 21 , 25 ].…”

Pulmonary Manifestations of Sjögren's Disease

“…It is pathologically characterized by the misfolding of extracellular proteins with deposition of protein in an abnormal fibrillar form. 68 It is classically identifiable as an amorphous, eosinophilic material which displays an apple-green birefringence under polarized light after Congo red staining. 69 The most common forms of amyloidosis are light-chain (AL) or reactive (AA) amyloidosis.…”

“…70 A heavy female predominance is observed among SjD patients with amyloidosis. 68,[71][72][73][74][75][76][77] Disease onset is typically in the fifth decade, and most patients have a pre-existing diagnosis of SjD when amyloidosis is diagnosed. Common symptoms include cough and dyspnea in over half of all patients, with a smaller proportion of asymptomatic incidental diagnoses.…”