Sjögren's syndrome is a slowly progressive autoimmune inflammatory disease that is associated with destruction of the exocrine glands (salivary glands) and can lead to the absence or reduction of secretions in many places in the body.
Sjögren's syndrome may, in some cases, be associated with systemic diseases involving the lungs, liver and kidneys.
Despite the fact that the incidence of pulmonary problems in the disease was described by Henrik Sjögren more than fifty years ago, it continues to be largely underdiagnosed even today. There are descriptions of patients with Sjögren's syndrome presenting with several pulmonary complications, including bronchial complications, bronchiolitis, interstitial pneumonia, and lymphoma.
The spectrum of pulmonary complications can be even wider if we consider that secondary Sjögren's syndrome can coexist with any other disease of the autoimmune system.
In the case of primary Sjögren's syndrome, the true frequency of pulmonary complications is not well defined.
Bronchitis and dry trachea (xerotrachea): The respiratory tract appears to be the most common target in patients with primary Sjögren's syndrome.
In one study, obstruction was noted in 25% of cases of primary Sjögren's syndrome observed. The obstruction of the small ducts in these cases is probably the result of infiltration by inflammatory cells similar to those found in the salivary glands.
The most frequently observed clinical occurrence linked to a problem with the respiratory tract is that of a dry cough which is present in 40% of patients and which can be of variable intensity. This cough is usually linked to the dryness observed in the bronchial tree which is due to the inability of the salivary glands of the bronchial tree to secrete sufficient mucus.
This abnormality due to inflammatory bronchitis in Sjögren's syndrome patients is rarely significant when observed clinically.
Diagnosis can also be complicated because chest imaging methods, such as X-rays, CT scans or others, often reveal nothing despite the fact that the small airways are effectively blocked. These methods have only proven effective in the most advanced cases.
The discovery of lymphocytes in patients with active Sjögren's syndrome is not limited to the bronchial tree, but is also found in other organs such as the liver or kidneys. The term autoimmune epithelitis, which was given by Dr. H. Moutsopoulos to explain the abnormalities of the salivary glands as well as other glandular tissues (involved in Sjögren's syndrome), comes from these discoveries made in the lungs and other major organs.
Sjögren's syndrome lies at the intersection of immune system disease and lymphoid malignancy. For some patients, the disease covers a broad spectrum of symptoms ranging from benign tissue infiltration to malignant lymphoid infiltration. In some patients, benign lymphoid tissue is observed. This may present as “follicular bronchiolitis” which can mimic interstitial lung disease. This form usually responds very well to treatment with corticosteroids.
Lymphoid interstitial pneumonia is a lung problem that infiltrates the tissues. This is a classic but uncommon phenomenon of primary Sjögren's syndrome and may also be linked to other viral infections and other chronic abnormalities such as liver disease. To make the diagnosis, a long biopsy is necessary and, here again, this condition responds well to steroid therapy, accompanied or not by immunosuppressive drugs. Primary lymphoma in SS cases is uncommon and presents as a solitary or diffuse image on chest X-ray. To make this diagnosis, a lung biopsy is also necessary.

Interstitial pulmonary fibrosis

Pulmonary interstitial fibrosis is rather rare in patients with Sjögren's syndrome. In one study, 2 out of 343 patients were found to suffer from this abnormality. This is often associated with progressive scarification of the lungs. For this entity, we proceed with experimental medication.
Other pulmonary diseases less common in S.S. Disease.
Other less common entities, such as vasculitis and primary pulmonary hypertension, which involves the circulation in the lungs, are also very rare in Sjögren's syndrome. Other forms of bronchiolitis have also been described in Sjögren's syndrome, but they are also very rare.

Summary

Henrik Sjögren was the first to describe the involvement of the lungs in Sjögren's syndrome more than 50 years ago. Since that time, the incidence of pulmonary involvement in primary Sjögren's syndrome has been the subject of numerous studies, and several pulmonary complications have been described including bronchial diseases, interstitial pneumonia and lymphoma. Although common and presenting in several forms, complications of this type are rarely severe, when observed clinically, in patients with Sjögren's syndrome.
If the patient suffers from significant shortness of breath and changes are observed on the X-rays, a cellular diagnosis is imperative, i.e. a transthoracic lung biopsy. In light of these results, treatments are generally effective when initiated at the beginning of the disease.
Source: Moisture Seekers, May 2001
Thanks to Madame Lefrançois from the Volunteer Center for the translation of this text.