The other connective tissue disease-associated interstitial lung diseases: Sjogren's syndrome, mixed connective tissue disease, and systemic lupus erythematosus

Abstract

Purpose of reviewWe review the clinical manifestations of three less common connective tissue disease (CTD)-associated interstitial lung diseases (ILDs): Sjogren's syndrome (SjS), mixed CTD (MCTD), and systemic lupus erythematosus (SLE). Recent findingsSjS is classically associated with lymphocytic interstitial pneumonia and cystic lung disease, but the most common type of ILD in Sjogren's patients is nonspecific interstitial pneumonia. ILD is prevalent in MCTD and associated with worse survival. SLE-associated ILD, while rare, is more common in those with CTD overlap syndromes. Regardless of underlying cause, a subset of patients with fibrotic CTD-associated ILD develop a progressive course for which antifibrotic agents and lung transplantation should be considered. SummaryAn understanding of the characteristics of ILD in SjS, MCTD, and SLE is important for the pulmonary specialist. Future research should identify risk factors for progression and develop additional treatment modalities for both CTD-related autoimmune features and progressive ILD.

Umbilical cord mesenchymal stem cells alleviate Sjögren’s syndrome and related pulmonary inflammation through regulating Vγ4+ IL-17+ T cells

“…Among them, SS-related complications in the lung are frequent, for example, pulmonary inflammation affects about 10-60% of SS patients (4,5). Moreover, SS-related pulmonary inflammation is a critical factor leading to poor prognosis in SS patients, with a 5-year cumulative mortality rate of up to 16% (6,7).…”

Unravelling the enigma of systemic lupus erythematosus‐associated ILD

“…In these trials, cardio‐respiratory outcome measures are broadly captured as part of global disease activity scores (such as the British Isles Lupus Assessment Group [BILAG] Index and Systemic Lupus Erythematosus Disease Activity Index [SLEDAI]); however, detailed information regarding pulmonary manifestations is not reported. What limited data are available suggest that ILD is less common in SLE compared to other CTDs, with estimates of clinically significant SLE‐ILD ranging from 3% to 8% 2, 3 . The most frequently reported radiologic patterns include non‐specific interstitial pneumonia, lymphocytic interstitial pneumonia and organizing pneumonia.…”

“…Disease trajectory is variable, often following a relapsing–remitting course, that is typically responsive to immunosuppressive therapy. In a minority of individuals, SLE‐ILD may present with catastrophic, potentially life‐threatening manifestations including acute lupus pneumonitis and diffuse alveolar haemorrhage 2, 3 …”

“…What limited data are available suggest that ILD is less common in SLE compared to other CTDs, with estimates of clinically significant SLE-ILD ranging from 3% to 8%. 2,3 The most frequently reported radiologic patterns include non-specific interstitial pneumonia, lymphocytic interstitial pneumonia and organizing pneumonia. Disease trajectory is variable, often following a relapsing-remitting course, that is typically responsive to immunosuppressive therapy.…”

“…In a minority of individuals, SLE-ILD may present with catastrophic, potentially lifethreatening manifestations including acute lupus pneumonitis and diffuse alveolar haemorrhage. 2,3 Understanding the extent of a problem is a necessary first step before developing a solution, and in a recent publication in Respirology, Mageau and colleagues provide an epidemiologic snapshot of ILD in one of the largest SLE cohorts ever reported. 4 Utilizing a nationwide hospital medical information database (Programme de Medicalisation des Systemes d'Informations, PMSI), the authors report the prevalence, incidence and mortality for SLE-ILD in hospitaladmitted patients over a 10-year period.…”