Sjögren Syndrome Complicated with Cystic Lung Disease and Pulmonary Amyloidosis

A 72-year-old Japanese woman was noted to have multiple cystic lung shadows and infiltrates on chest radiography and computed tomography (CT). She complained of dryness of the mouth and eyes, but she did not have respiratory symptoms, such as cough, sputum production, and dyspnea. Her laboratory findings showed high titers of anti-SSA/Ro and anti-SSB/La antibodies. Surgical lung biopsy was performed and demonstrated pathologic findings of amyloid light-chain deposition and bronchiolitis with lymphocytic infiltration. Taken altogether, she was diagnosed as Sjögren syndrome with bronchiolitis and pulmonary amyloidosis. Since then, she has been carefully followed up without treatment. After 6 years, the cystic lung lesions on CT gradually enlarged and increased in number, but she remained to have no respiratory symptoms and no manifestations of lymphoma. Here, we report a rare case of Sjögren syndrome complicated with cystic lung disease and pulmonary amyloidosis.

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Cystic Lung Disease Among Patients With Sjögren Syndrome

CHEST Journal ◽

10.1016/j.chest.2016.05.015 ◽

2016 ◽

Vol 150 (3) ◽

pp. 631-639 ◽

Cited By ~ 8

Author(s):

Carlos D. Martínez-Balzano ◽

Sunkaru Touray ◽

Scott Kopec

Keyword(s):

Lung Disease ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Cystic Lung ◽

Cystic Lung Disease

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Diffuse Cystic Lung Disease: Consider Sjögren Syndrome in the Differential Diagnosis

The American Journal of Medicine ◽

10.1016/j.amjmed.2020.01.028 ◽

2020 ◽

Vol 133 (8) ◽

pp. e438-e439

Author(s):

Luis Alonso González ◽

Adelis Enrique Pantoja-Marquez ◽

Philip Johnson

Keyword(s):

Differential Diagnosis ◽

Lung Disease ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Cystic Lung ◽

Cystic Lung Disease

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Diffuse Cystic Lung Disease as the Presenting Manifestation of Sjögren Syndrome

Annals of the American Thoracic Society ◽

10.1513/annalsats.201511-759bc ◽

2016 ◽

Vol 13 (3) ◽

pp. 371-375 ◽

Cited By ~ 10

Author(s):

Nishant Gupta ◽

Kathryn A. Wikenheiser-Brokamp ◽

Aryeh Fischer ◽

Francis X. McCormack

Keyword(s):

Lung Disease ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Cystic Lung ◽

Cystic Lung Disease

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CT-guided biopsy in the differential diagnosis of Sjogren syndrome associated cystic lung disease: A case of lung nodular AL-k amyloidosis

Radiology Case Reports ◽

10.1016/j.radcr.2020.08.012 ◽

2020 ◽

Vol 15 (11) ◽

pp. 2331-2334

Author(s):

Claudio Tirelli ◽

Giovanni Zanframundo ◽

Adele Valentini ◽

Chandra Bortolotto ◽

Roberto Dore ◽

...

Keyword(s):

Differential Diagnosis ◽

Lung Disease ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Ct Guided ◽

Cystic Lung ◽

Cystic Lung Disease ◽

Guided Biopsy ◽

Ct Guided Biopsy

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Pulmonary amyloidosis and cystic lung disease in primary Sjögren’s syndrome: a case report and literature review

Clinical Rheumatology ◽

10.1007/s10067-021-05596-9 ◽

2021 ◽

Author(s):

Haiyun Li ◽

Yuewu Lu

Keyword(s):

Case Report ◽

Literature Review ◽

Lung Disease ◽

Sjogren's Syndrome ◽

Primary Sjögren’S Syndrome ◽

Primary Sjogren’S Syndrome ◽

Primary Sjögren's Syndrome ◽

Cystic Lung ◽

Cystic Lung Disease ◽

Pulmonary Amyloidosis

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Chronic Obstructive Pulmonary Disease Is Common in Never-smoking Patients with Primary Sjögren Syndrome

The Journal of Rheumatology ◽

10.3899/jrheum.140370 ◽

2015 ◽

Vol 42 (3) ◽

pp. 464-471 ◽

Cited By ~ 9

Author(s):

Anna Matilda Nilsson ◽

Sandra Diaz ◽

Elke Theander ◽

Roger Hesselstrand ◽

Eeva Piitulainen ◽

...

Keyword(s):

Chronic Obstructive Pulmonary Disease ◽

Cigarette Smoking ◽

Disease Activity ◽

Lung Disease ◽

Respiratory Symptoms ◽

Sjögren Syndrome ◽

Chronic Obstructive ◽

Sjogren Syndrome ◽

Obstructive Pulmonary Disease ◽

Primary Sjögren Syndrome

Objective.To assess the prevalence of chronic obstructive pulmonary disease (COPD) in patients with primary Sjögren syndrome (pSS) and to study the association of COPD with cigarette smoking, radiographic features, respiratory symptoms, disease activity, and laboratory inflammatory and serological features in patients with pSS.Methods.Fifty-one consecutive patients with pSS (mean age 60 yrs, range 29–82 yrs, 49 women) were assessed by pulmonary function tests (PFT). The PFT results were compared with previously studied population-based controls, standardizing results with regard to sex, age, height, weight, and cigarette smoking. In addition, patients with pSS were assessed by computed tomography of the chest, the European League Against Rheumatism Sjögren Syndrome Disease Activity Index and Patient Reported Index, the St. George’s Respiratory Questionnaire (which evaluates respiratory symptoms), and by laboratory inflammatory and serological tests.Results.Forty-one percent of all patients with pSS and 30% of the never-smoking patients with pSS fulfilled the Global Initiative for Chronic Obstructive Lung Disease criteria for COPD. Vital capacity (VC), forced expiratory volume in 1 s (FEV1), FEV1/VC ratio, and DLCO were significantly decreased while residual volume (RV) and the RV/total lung capacity ratio were significantly increased in patients with pSS. Moderate correlations between PFT results, symptoms, and disease activity were found. However, laboratory inflammatory and serological features were poorly associated with PFT results in patients with pSS.Conclusion.COPD was a common finding in patients with pSS, even among never-smoking patients. An obstructive pattern was the predominant PFT finding in patients with pSS, although a superimposed restrictive lung disease could not be excluded. The results suggest that the disease per se is involved in the development of COPD in pSS.

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