Nodular Pulmonary Amyloidosis: A Manifestation of Sjögren’s Syndrome

Abstract

Case Description A 72-year-old woman with primary Sjögren Syndrome (SS) was diagnosed during an inpatient hospital stay with dry symptoms. The patient had respiratory and constitutional symptoms, requiring a pulmonary imaging evaluation by high-resolution computed tomography (HRCT) of the thorax. Clinical Findings Multiple cavitary pulmonary nodules, halo sign, and focal areas of ground-glass opacity with predominance in both bases were found in the images. Complementary studies were done where a neoplastic focus was ruled out. The diagnosis of nodular pulmonary amyloidosis was confirmed with a pulmonary biopsy performed by videothoracoscopy for histopathological study, which reported the formation of nodules in the parenchyma with amyloid deposits and positive immunohistochemical markers for CD3, CD20, and CD38 lymphocytic infiltration. Treatment and Outcome Initial inpatient management with intravenous cyclophosphamide and methylprednisolone was given. Subsequent outpatient management was given with high dose glucocorticoids. Clinical Relevance We presented a case of nodular pulmonary amyloidosis in a female patient with primary SS, which is a rare pulmonary manifestation of this syndrome.

A spectrum of Thymic mucosa-associated lymphoid tissue lymphoma and Thymic amyloidosis in the patient with Auto immune disease: a case series

“…These findings led to diagnosis of thymic MALT lymphoma with amyloid deposition. Lung cysts were bullae with amyloid deposits, consistent with lung manifestation of SjS ( 6 , 7 ).…”

An unusual case of nodular pulmonary amyloidosis

“… 4 Most of the patients are asymptomatic, but in some cases, symptoms such as wheeze, stridor, cough, and recurrent pneumonia are present. 6 The disease should be considered in patients with multiple lung nodules. In the presented case, only a single nodule was present, which is an extremely rare presentation of amyloidosis referred to as amyloidoma.…”

“…The differential diagnosis for nodular pulmonary amyloidosis includes both malignant and benign primary lung neoplasms, such as bronchogenic carcinoma and mucinous adenocarcinoma. 6 The differential diagnosis could also include fungal or tubercular granulomas. 6 However, in both tumor and infection, rapid progression would be observed, which was not the case for this patient.…”

“… 6 The differential diagnosis could also include fungal or tubercular granulomas. 6 However, in both tumor and infection, rapid progression would be observed, which was not the case for this patient.…”

Expression of Matrix Metalloproteinase-2, Matrix Metalloproteinase-9, Tissue Inhibitor of Metalloproteinase-1, and Changes in Alveolar Septa in Patients with Chronic Obstructive Pulmonary Disease

“…With the increase of alveolar septa thickness, the DLCO%pre and FEV 1 %pre levels were decreased, and FEV 1 %pre was decreased significantly. The reason can be that the alveolar septum is the nodular tissue between adjacent alveoli, which contains a capillary network, and the thickening of the alveolar septum will inevitably lead to an increase in the gas diffusion distance and reduce the pulmonary function of patients with COPD [ 31 , 32 ]. Furthermore, collagen fibers were increased in patients with COPD, and collagen fibers in the severe COPD group were significantly increased compared with that of the mild and moderate groups.…”