Background/Purpose: Pulmonary involvement is common in primary Sjögren’s syndrome (pSS). Clinicopathologic pulmonary manifestations associated with pSS have yet to be reviewed in a large series. However, the pulmonary functional and radiological characteristics of pSS-associated lung disease which are more practical in clinic were less studied. Besides, the treatment of those patients has hardly been explored before. Most studies were specifically designed to evaluate sicca features. We aimed to describe the clinical, radiologic, and pulmonary Functional characteristics in a large Chinese pSS patient with lung involvement, and to analyze efficacy and safety of corticosteroid therapy combined with hydroxychloroquine (HCQ) or intravenous cyclophosphamide (CTX) in pSS-associated lung disease.

Methods: A total of 112 hospitalized patients with pSS-associated lung disease were retrospectively analyzed. The high-resolution computed tomography (HRCT) was re-evaluated by two experienced chest radiologists. Autoantibodies, inflammation markers,arterial blood gas (ABG) and PFT results were obtained. A total of 15 patients were recruited in the prospective study. Prednisone was prescribed initially at a dosage of 30-40mg/day, and was tapered to 7.5mg/d within 4 months. HCQ was administered at a dosage of 200mg, twice a day. CTX was administered intravenously at an initial dosage of 400mg, every 2 weeks for 6 months, and then tapered to 400mg every 4 weeks.

Results: Among the 112 pSS patients with lung involvement, 102 (91.10%) were female. The mean age was 61.74±10.24 years old. The disease duration was 60 months (12.00 to 147.00 months). Elevated IgG was prominent in those patients. There were more frequent and severer of the lower lung lobes involvement. The most frequent HRCT findings were linear opacities (94.2%), ground-glass attenuation (87.0%), reticular pattern (65.2%) and pleural involvement (65.2%). Impaired diffusing capacity was the most significant (74.3%). Among the 36 patients who took ABG, 20 patients had hypoxia and 7 had Type 1 respiratory failure.

Seven patients took oral prednisone combined with HCQ and 8 patients received oral prednisone combined with intravenous CTX. One patient from CTX group withdrew from the study for stopping DMARDs to have surgery. The rest were followed up for 11.57±4.91 months. There was evidence of improvement in HRCT for most of the patients, and no obvious deterioration of PFT was observed. One patient in HCQ group presented hypoleukemia, which was ameliorated after reducing to 100mg, twice a day. Two patients had pneumonia and one had herpes zoster virus (HZV) infection in CTX group.

Conclusion: The distribution of the abnormalities and severe parenchymal involvement are most pronounced in the lower lobes of pSS patients. Impaired diffusing capacity is the most significant PFT abnormalities. Hypoxia is not rare for pSS-association lung involvement patients. Corticosteroid therapy combined with hydroxychloroquine or intravenous cyclophosphamide is administered with a favorable response seen in the majority of patients. This should be further confirmed in a large cohort.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-manifestations-and-treatment-of-primary-sjogrens-syndrome-associated-lung-involvement-patients-a-prospective-study/