Interstitial lung disease in Sjögren's syndrome-Importance, treatment strategies

Sj & ouml;gren's syndrome or Sj & ouml;gren's disease (SjD) is the most frequent connective tissue disease with a prevalence of up to 1:200. Approximately 10-20% of patients suffer from interstitial lung disease (ILD), which can be the first symptom of SjD and limits the prognosis. The supposed cardinal symptom of SjD of ocular and oral dryness is unspecific. The diagnostic work-up therefore requires objectivization of the glandular dryness as well as serological testing for antibodies against Ro/SSA or, in the absence of this biomarker a biopsy of small salivary glands. Testing for antibodies against Ro/SSA should at least be performed for newly diagnosed ILD. The biopsy of small salivary glands is meaningful in patients with ILD and without Ro/SSa antibodies when no usual interstitial pneumonia (UIP) pattern with computed tomography (CT) or hypergammaglobulinemia or an objective glandular dryness is present. For the treatment of ILD in SjD immunosuppressants, such as mycophenolate mofetil, azathioprine, calcineurin inhibitors or cyclophosphamide as well as b-cell depleting biologicals, such as rituximab are recommended. In progressive fibrosing ILD, antifibrotics are also used.