Interstitial Lung Disease in Mixed Connective Tissue Disease: An Advanced Search

Abstract

The spectrum of pulmonary manifestations associated with mixed connective tissue disease ranges from pulmonary hypertension and interstitial lung disease to pleural effusions, alveolar hemorrhage, and complications from the thromboembolic disease. Interstitial lung disease in mixed connective tissue disease is a frequently occurring entity, although in most cases it tends to be self-limited or slowly progressive. Despite this, a significant percentage of patients may present a progressive fibrosing phenotype, thus posing a great challenge regarding its therapeutic approach, given the scarcity of clinical studies that compare the efficacy of immunosuppressants available to date. Due to this, many recommendations are extrapolated from other diseases with similar characteristics such as systemic sclerosis and systemic lupus erythematosus. That is why it is proposed to carry out an advanced search of the literature in order to clarify its clinical, radiological, and therapeutic characteristics to achieve its evaluation from a holistic point of view.

Clinical presentation, course, and prognosis of patients with mixed connective tissue disease: A multicenter retrospective cohort

“…As in our study, the main reported causes of death are ILD and PH [19,24,25,29]. In MCTD, ILD occurs in 25%-52% patients [17,24,29,32,33] and is a matter of concern as it is responsible for pulmonary function impairment, reduced physical capacity [17], and increased mortality [39][40][41]. Regarding PH, our group has recently reported a retrospective study of 36 well-characterized patients highlighting that PH may reveal MCTD in up to a quarter of the patients, resembles SLE-and SSc-related PH, and is associated with poor survival [42].…”

“…Baseline clinical and biological characteristics are detailed in Tables 1 and 2. A total of 291 patients (88.2%) were females with a median (IQR) age at diagnosis of 35 (26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41)(42)(43)(44)(45) years. A total of 208 (63.0%) patients were Caucasian, 97 (29.4%) of Afro-Caribbean origin, and 21 (6.4%) of Asian origin.…”

“…Results. Three hundred and thirty patients (88% females, median [interquartile range] age of 35 years [26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45]) were included. The diagnostic criteria of Sharp or Kasukawa were met by 97.3% and 93.3% of patients, respectively.…”

Fractional exhaled nitric oxide in idiopathic pulmonary arterial hypertension and mixed connective tissue disease complicating pulmonary hypertension

“…MCTD-PH may be associated with interstitial lung disease (ILD), and recent literature has increasingly emphasized the significant role of ILD as a contributor to morbidity and mortality among patients with MCTD-PH [ 38 ]. To mitigate the potential influence of ILD on FeNO and lung function outcomes, we excluded data from four patients with MCTD who also presented with ILD, the conclusions have not changed (the data were not listed in the table), it suggests that the presence of ILD did not significantly affect the FeNO in severe MCTD-PH patients in the manuscript.…”