Background/Purpose:

Various forms of interstitial lung disease (ILD) are associated with primary Sjögren’s syndrome.  IgG4-related sclerosing disease (ISD) is a recently described systemic fibroinflammatory disease associated with dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells. Sicca symptoms and ILD are two of the known manifestations of ISD, and are also characteristic of Sjögren’s syndrome. We aimed to describe the lung histopathology in a series of patients with Sjögren’s syndrome who underwent open lung biopsy for evaluation of ILD, and to evaluate the occurrence of IgG4 in the lung parenchyma from a subset of these patients.

Methods:

The archived pathology specimens of 15 patients with primary Sjögren’s syndrome related ILD who underwent lung biopsies between 1998 and 2010 were reviewed, and evaluated for the presence of IG4 by immunohistochemical staining. Diagnosis was based on 2002 American European Consensus Group criteria for primary Sjogren’s syndrome. Patient demographic data and findings from high resolution computed tomography (HRCT) findings of the lungs of these patients were recorded.   

Results:

Among the 15 identified patients, 11 (73%) were female; mean age was 58 years. All of these patients had dry eyes and dry mouth symptoms. Anti-nuclear antibodies were present in 11 (85%) of 13 tested patients. Of 12 patients tested, 12 (100%) had anti- SSA antibodies and 9 (75%) had anti-SSB antibodies. Six (55%) of 11 tested patients were rheumatoid factor positive, and  hypergammaglobulinemia was noted in 9 (75%) of 12 tested patients. Six (40%) patients had positive smoking history. Lung histopathology revealed usual interstitial pneumonia (UIP) in 6 patients; 3 patients had non-specific interstitial pneumonia (NSIP). The other patients had bronchiolitis (n=1), cryptogenic organizing pneumonia (n=1), desquamative interstitial pneumonia (n=1), follicular bronchiolitis with amyloid (n=1), obstructive pneumonitis (n=1), and lymphocytic interstitial pneumonia (n=1). Immunohistochemical staining of these lung biopsy specimens for IgG4 were negative for 7 (100%) of 7 patients. HRCT was performed in 12 of 15 patients. Linear or reticular opacities were noted in 9 patients, ground glass or non-solid opacities in 8 patients, and bronchiectasis in 8 patients. Other common HRCT findings were lymphadenopathy (n=3) honey-combing (n=3), multifocal cysts (n=3) and solid nodules (n=3).

Conclusion:

The most common form of ILD in these patients with Sjögren’s syndrome undergoing lung biopsy is UIP, and the majority had linear and/or ground opacities on HRCT. While there are reports that dacroadenitis and sialoadenitis is associated with IgG4 deposition, we found no evidence of IgG4 in the lung tissue of patients with primary Sjögren’s syndrome suffering from ILD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/interstitial-lung-disease-in-primary-sjogrens-syndrome-any-association-with-igg4-related-sclerosing-disease/