Background/Purpose: Mixed connective tissue disease (MCTD) is an immune-mediated systemic disorder characterized by serum autoantibodies against U1-ribonucleoprotein and diverse multisystemic clinical manifestations. Approximately 50% of patients with MCTD develop a radiological pattern of interstitial lung disease (ILD). In this study, we explore the phenotypic and clinical features in patients with MCTD that are associated with development of ILD.

Methods: We performed a retrospective case control study utilizing data from patients evaluated at a single tertiary care center from 2007-2014. Twenty-eight patients who met validated criteria for diagnosis of MCTD were included in the study. Fourteen patients had high-resolution computed tomography or biopsy-proven ILD, and 14 had MCTD without evidence of ILD . We performed a multivariate logistic regression with multiple demographic, clinical, and serologic predictor variables, and ILD as the outcome variable.

Results:  Two clinical variables had an association with development of ILD in patients with MCTD:

1. Dysphagia with a R² value of 0.33 (p value < 0.001)
2. Raynaud’s phenomenon with R² value of 0.28 (p value < 0.001)

 We did not find a significant association between any other demographic, clinical, or serologic variables and development of ILD in patients with MCTD.

Conclusion: Dysphagia is one of the symptoms of esophageal involvement in patients with autoimmune connective tissue disorders. An association of dysphagia with the development of ILD in our study is in harmony with the existing literature, wherein esophageal dysmotility has been described in a cluster of patients with MCTD and ILD. Scant data, primarily case reports, suggest an association of Raynaud’s phenomenon with development of ILD in patients with undifferentiated CTD. To our knowledge, this is the first study highlighting the association of Raynaud’s phenomenon with development of ILD in patients with MCTD.   This study is limited by its small size and retrospective nature. The mechanistic aspects of the association between Raynaud’s phenomenon and ILD remain unexplored. The association of easily elicited historical and clinical features of MCTD with subtle, but worrisome, pulmonary pathology carries the promise of sensitizing the unsuspecting clinician about the entity of ILD in MCTD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-predictors-for-development-of-interstitial-lung-disease-in-mixed-connective-tissue-disease/