In women with interstitial lung disease (ILD) that is secondary to an autoimmune disorder, the risk for maternal mortality and pregnancy-related cardiorespiratory adverse events is low overall, according to results of a retrospective medical records review recently published in the journal Arthritis Care & Research (Hoboken).

Clinicians often recommend that women with ILD avoid pregnancy based on the limited existing data on ILD and pregnancy. Researchers thus sought to establish maternal and pregnancy outcomes in the largest-to-date cohort of women with the disease. Patients included in the analysis were found through a review of medical records from the Duke University Health System in Durham, North Carolina to locate pregnant women with a diagnosis of ILD who had an underlying autoimmune disease. Pregnancies reviewed were classified as having very severe, severe, mild to moderate, or normal lung function, based on the results of pulmonary function tests (PFTs).

Composite variables from the Predictors of Pregnancy Outcomes in Systemic Lupus Erythematosus and Antiphospholipid Syndrome (PROMISSE) study were used to measure adverse pregnancy outcomes (APOs). PROMISSE-APO criteria included singleton pregnancies with an outcome of neonatal death, fetal death at more than12 weeks, preterm delivery at less than 36 weeks, preeclampsia, and small for gestational age (<5th percentile). Severe PROMISSE-APO criteria included singleton pregnancies with an outcome of neonatal death, fetal death at greater than 12 weeks, preeclampsia and preterm delivery at less than 34 weeks, or preterm delivery at less than 30 weeks. Termination of a pregnancy at greater than 12 weeks was considered to be a fetal death in the APO analysis.

A total of 86 pregnancies in 60 unique women were included in the current study. Overall, 59 of the pregnancies had PFTs available for classification of ILD severity, with 12% (7 of 59) of the pregnancies in women with very severe disease, 25% (15 of 59) in patients with severe ILD, 51% (30 of 59) in participants with mild to moderate disease, and 12% (7 of 59) in those with normal PFTs. Among the patients with very severe ILD, 1 with very severe ILD experienced a pre-pregnancy oxygen requirement (ie, 5 L/min). The woman only needed to use oxygen with exertion, however, and did not require the use of supplemental oxygen at delivery.

PROMISSE-APO was reported in 32% of the pregnancies, which included all pregnancies in women with very severe ILD (P =.02 across severity groups); 56% of the pregnancies with CTD-ILD; and 23% of the pregnancies with sarcoidosis (P =.02). Severe PROMISSE-APO was observed in 15% of the pregnancies, which included 60% with very severe ILD and 28% with CTD-ILD. 

Among this cohort of patients, 71% of the pregnancies were in women with a diagnosis of sarcoidosis, with 67% of them having biopsy-proven disease. The remaining 29% of the pregnancies had connective tissue disease–associated ILD (CTD-ILD), which was characterized by the following underlying disorders: systemic lupus erythematosus in 11 women; polymyositis in 3 patients; scleroderma in 3 participants; Sjögren’s syndrome in 2 individuals; undifferentiated CTD in 2 women; eosinophilic granulomatosis with polyangiitis in 2 patients; granulomatous polyangiitis in 1 participant; and lymphocytic interstitial pneumonia in 1 woman. In all of the pregnancies among those with very severe disease, the women also had a diagnosis of CTD-ILD. Overall, 86% of the pregnancies in patients with normal PFTs had a diagnosis of sarcoidosis (P =.002 across groups).

The investigators concluded that the findings from this study show that pregnancies in women with ILD, particularly when accompanied by significant rheumatic disease or very severe lung disease, can still have significant adverse pregnancy-related outcomes. These data do offer hope to women with ILD who are contemplating pregnancy, provided that: 1) their condition is well controlled on pregnancy-compatible medications; 2) they receive preconception assessment and counseling; and 3) they are monitored closely during their pregnancy and following delivery.

Disclosure: Some of the study authors have declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of disclosures.

Reference  

Clowse MEB, Rajendran A, Eudy A, Giattino S, Swaminathan A, James AH. Pregnancy outcomes in patients with interstitial lung diseaseArthritis Care Res (Hoboken). Published online November 8, 2021. doi:10.1002/acr.24814