ABSTRACT
Background: Antisynthetase syndrome (ASyS) is a rare autoimmune disease characterized by inflammatory myopathy, arthritis, fever, and interstitial lung disease (ILD). Pulmonary involvement in ASyS significantly increases morbidity and mortality and, therefore, requires prompt and effective immunosuppressive treatment. Owing to the rarity of ASyS, limited data exists on progression and prognosis of ILD under immunosuppression. Objectives: The objective of the study was to evaluate the radiological progression and outcome measures of ILD with immunosuppressive therapy in patients with ASyS. Methods: Twelve patients with ASyS-associated ILD (ASyS-ILD) were included. Demographic and clinical data, including organ involvement, pulmonary function tests (PFT), laboratory parameters, imaging studies, and treatment regimens were retrospectively analyzed from routinely collected data. The extent of ground glass opacities, fibrotic changes and honeycombing was analyzed and scored using high-resolution chest computed tomography (HRCT) scans. HRCT findings were compared between baseline and follow-up examinations. In addition, patients were stratified depending on whether they had received rituximab (RTX) or not. Results: Pulmonary function tests revealed stable lung function and follow-up HRCT scans showed an improvement of radiological alterations in the majority of ASyS patients under immunosuppressive therapy. We did not detect significant differences between the RTX- and non-RTX-treated groups, but the RTX-treated patients more frequently had myositis and relapsing disease. Conclusions: Radiographic alterations in ASyS-associated ILD respond to immunosuppressive treatment. RTX is a feasible treatment option with similar clinical and radiographic outcomes in patients with relapsing disease and clinically apparent myositis.
ABSTRACT
Proton pump inhibitors are among the most commonly used drugs worldwide. They are considered to be largely safe and cause little side-effects. We report a 69-year-old woman who suffered from erythematous plaques 2 months after initiating therapy with esomeprazole. The diagnosis of subacute cutaneous lupus erythematosus was based on the clinical picture together with characteristic histological features of a skin biopsy specimen and the detection of anti-Ro/SSA antibodies. In particular, the temporal relationship with the onset of proton pump inhibitor therapy led to the high-level suspicion of a drug-induced pathogenesis. Strengthening the initially suspected diagnosis, the characteristic skin lesions resolved almost completely without specific therapy after discontinuation of the medication. If typical skin lesions occur in light-exposed areas in connection with the administration of PPI, the possibility of a drug-induced cutaneous lupus erythematosus should always be considered. Due to the frequent recurrence rate after renewed exposure, a new therapy with PPI should be avoided.
