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A1541 - Surfactant Protein C Deficiency and Rheumatoid Arthritis Interstitial Lung Disease in Pregnancy - A Fatal Combination
Author Block: L. Barnes1, P. J. Passarelli2, R. Hsiao3, R. E. Sell4; 1University of California San Diego, Internal Medicine, San Diego, CA, United States, 2Internal Medicine-Pediatrics, UC San Diego, San Diego, CA, United States, 3University of California San Diego, San Diego, CA, United States, 4Univ of CA-San Diego, San Diego, CA, United States.
Introduction:
Rheumatoid Arthritis - Interstitial Lung Disease (RA-ILD) is usually tolerated in pregnancy. Surfactant protein C gene mutation (SFPTC) associated ILD is rare with no published case reports during pregnancy. We present a case of SFPTC and RA-ILD during pregnancy.
Case Description:
A 24-year-old pregnant woman with a history of Juvenile RA and SFPTC with ILD with restrictive lung disease had been followed since age 12. She was first diagnosed with ILD and SFPTC by lung biopsy, which showed severe chronic interstitial inflammation with bronchiectasis, active bronchiolar fibrosis, and areas of vasculitis. Her mother and brother likely also had SFPTC, and her sister had recently died from the disease. Her symptoms were generally well controlled until, despite being advised to use contraception, the patient became pregnant. At 31 weeks of gestation she was seen in Pulmonology clinic and noted to be hypoxic. She was sent to the Emergency Department and was admitted to the Obstetric service. Three days into admission, the patient was intubated for progressive hypoxic respiratory failure, thought to be from a flare of her pulmonary vasculitis, and was taken for emergent Cesarean section. She required extracorporeal membrane oxygenation for six days. Her hospital course was complicated by multiple infections, including klebsiella and pseudomonas pneumonia, cytomegalovirus colitis and pneumonitis. Despite multiple rounds of steroids with transient improvement, broad-spectrum antibiotics, antivirals, and diuresis, the patient was unable to be extubated. Unfortunately, she had a cardiac arrest in the setting of refractory hypoxemia and died on hospital day 66. Her son was healthy and tested negative for SFPTC.
Discussion:
SFPTC-associated ILD is a rare and poorly understood condition. Age of onset and severity of disease varies widely. This patient was unfortunate to also have Juvenile RA-associated ILD. ILD is relatively rare in pregnancy since the onset of most diseases occurs after the childbearing age. The effect of pregnancy on ILD is variable. For instance, it appears to positively affect sarcoidosis and worsen lymphangioleiomyomatosis. Its influence on SFPTC is unknown. It is unclear to what degree the hormonal, immunogenic, or physiological changes of pregnancy affect ILD. Furthermore, optimal management of acute respiratory failure in pregnant women is not clear. More needs to be learned about the effect of pregnancy on both these diseases.