Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome

Abstract

Sjogren's syndrome (SS) is a chronic autoimmune disease characterized by mononuclear cells (principally lymphocytes) infiltrating exocrine glands (e.g., salivary and lacrimal glands), leading to destruction of exocrine epithelial cells and dryness of mucosal surfaces. Cardinal symptoms are dry eyes (xerophthalmia) and dry mouth (xerostomia). Extraglandular sites are affected in 30 to 40% of cases of SS (particularly neurological, kidneys, skin, and lungs). B cell hyperactivity, autoantibody production, and hypergammaglobulinemia are cardinal features of SS. Primary SS is not associated with other autoimmune diseases. However, SS can complicate diverse autoimmune disorders (particularly systemic lupus erythematosus, rheumatoid arthritis, and scleroderma); this form is termed “secondary SS.” Pulmonary involvement is usually not a dominant feature of SS, but may be severe in some cases. In this review, we discuss specific tracheal, bronchiolar, and pulmonary complications of SS including xerotrachea, bronchiolitis, bronchiectasis, interstitial lung disease, nonspecific interstitial pneumonia, usual interstitial pneumonia, lymphoid interstitial pneumonia, organizing pneumonia, acute fibrinous and organizing pneumonia, pulmonary cysts, pleural effusions, pulmonary amyloidosis, and bronchus- or lung-associated lymphomas.

Diagnosis and management of lung involvement in systemic lupus erythematosus and Sjögren’s syndrome: a literature review

“… 130 It generally appears as a solitary nodule or mass on HRCT. 208 If mediastinal lymphadenopathy or the presence of pleural effusions is noted, a diagnosis of lymphoma should be considered, and histological confirmation is mandatory. Pseudolymphoma generally regresses after treatment with GCs or immunosuppressive therapy but rarely it progresses to lymphoma.…”

Bronchiolitis and Bronchiolar Disorders

“…Bronchiolar complications of CTDs are less well recognized than other pulmonary complications (e.g., ILD and pleural disease) of these disorders and occur most commonly in females with RA or Sjogren's syndrome, 74,75 less frequently in lupus erythematosus, ankylosing spondylitis, and systemic sclerosis (SSc). 76 Pulmonary function tests (PFTs) usually show poorly reversible airflow limitation that may be preceded by distal airflow limitation 77 ; in RA patients, factors significantly associated with abnormal forced expiratory flow between 25 and 75% of forced VC (forced expiratory flow, FEF25-75%) are respiratory symptoms, smoking history, and RA duration. 78 Patients with CTD-related bronchiolitis may have lower FEV1 when histology shows constrictive bronchiolitis compared with patients with nonconstrictive bronchiolitis (such as cellular or follicular); however, the rate of change of FEV1 does not seem to vary significantly in either subgroup over time.…”

“…Bronchiectasis detected by HRCT is found in approximately 30% of the patients with RA and less frequently in patients with other collagen-vascular diseases. 77,81 Histology findings in these patients are heterogeneous: follicular bronchiolitis, lymphocytic bronchiolitis without evident lymphoid follicles, centrilobular clusters of foamy macrophages, constrictive bronchiolitis, and acute epithelial injury often coexist in the same specimen 74,75 (►Fig. 6); cases of diffuse panbronchiolitis (DPB) have been described in Japanese patients with RA.…”

Pulmonary Involvement in a Mouse Model of Sjögren’s Syndrome Induced by STING Activation

“…In addition to exocrine dysfunction, pulmonary involvement occurs in up to 20% of SS patients, and it is a significant cause of mortality in SS [ 20 , 21 , 22 ]. However, a comprehensive evaluation with advanced imaging modalities like High-resolution CT scans and pulmonary lung function tests suggest a prevalence of subclinical disease in up to 58% of SS patients [ 23 ].…”