721 - Interstitial Lung Disease Secondary to Antisynthetase Syndrome in Acute Inpatient Rehabilitation: A Case Report

Thursday, February 22, 2024

5:00 PM – 6:30 PM EDT

Presenting Author(s)

Nicole C. Feng, MD

Resident Physician
Shirley Ryan AbilityLab/Northwestern McGaw Medical Center
Chicago, Illinois, United States

Co-Author(s)

JC

Joshua M. Chan, DO, MS

Resident Physician
UCI Health
Chino Hills, California, United States
VN

Victoria Nguyen, DO

Resident Physician
Shirley Ryan AbilityLab/Northwestern McGaw Medical Center
Chicago, Illinois, United States
CR

Christopher D. Reger, MD

Attending Physician
Shirley Ryan AbilityLab/Northwestern McGaw Medical Center
Chicago, Illinois, United States

Case Diagnosis: A woman with persistent acute hypoxemic respiratory failure after pneumonia is found to have interstitial lung disease secondary to antisynthetase syndrome.

Case Description: A 59 year old woman with a history of obesity, type 2 diabetes, and no prior pulmonary history was diagnosed with pneumonia four weeks prior and completed three courses of antibiotics and steroids without improvement. She returned to acute care with tachypnea, oxygen saturation of 50% on room air, and without a fever. A CT of the chest demonstrated findings suggestive of pulmonary fibrosis. Acutely, she required high flow nasal cannula 50L/50-70% FiO2. Additional diagnostic evaluation was notable for a negative infectious workup, antinuclear antibody 1:80, dsDNA negative, rheumatoid factor weakly positive at 17, anti-PL-7 strongly positive at 98, and anti-Ro/SSA positive. Despite no extrapulmonary manifestations, the most consistent clinical diagnosis was rapidly progressive interstitial lung disease secondary to antisynthetase syndrome. Pulse dose steroids, IVIG, and rituximab were initiated with improvement of supplemental oxygen needs to two liters of nasal cannula.

Discussions: Patient’s functional history was notable for independence with all ADLs. On admission to acute inpatient rehabilitation, she had impaired cardiovascular endurance with frequent desaturations, requiring close supervision and rolling walker with wheelchair follow at breaks of 80 feet. Frequent rest breaks were also required with full body dressing and toilet transfers. Prior to discharge after a two-week hospitalization, she had increased activity tolerance, able to ambulate 220 feet with standing rest breaks, negotiated four stairs, and improved endurance with dressing and transfers. Oxygen requirement remained at two liters of nasal cannula although desaturations improved with endurance.

Conclusions: Antisynthetase syndrome is an uncommon disease and up to 60% of patients develop interstitial lung disease. Acute inpatient rehabilitation can offer an important role in helping these patients reach functional goals.