Case Presentation: A 72-year-old female with rheumatoid arthritis, ILD, hypertension, hypothyroidism, iron deficiency anemia, and monoclonal paraproteinemia presented with 3-day history of progressive dyspnea and productive cough and was admitted for acute hypoxic respiratory failure initially requiring 3 L/minute oxygen via nasal cannula (NC). The remaining vital signs were stable. Exam revealed rales at right lower field. Initial labs were significant for respiratory alkalosis, lactic acidosis, anemia, elevated alkaline phosphatase and D-dimer, and no leukocytosis. Chest x-ray and CT showed chronic underlying ILD with superimposed infiltrates suspicious for infection with no evidence of pulmonary embolism. Given these findings, empiric antibiotics, low-dose steroids and one dose of diuretic were administered upon admission.After 48 hours, the patient’s respiratory status declined. Despite high-flow oxygen and BiPAP, increased dose of steroids, broadened antibiotics, and a daily diuretic, the patient’s status remained unchanged for the next 3 days. Echocardiography on day 7 revealed reduced systolic function (LVEF 35-40%) and mild enlargement of right and left atria compared to a normal exam 7 months prior. Goal-directed medical therapy was then initiated with metoprolol and dapagliflozin. In 48 hours, the patient was weaned off BiPAP but remained on high oxygen needs (8-9 LPM). Pulse doses of corticosteroids were initially withheld due to the history of severe psychosis presumably induced by steroids during the previous hospitalization for ILD exacerbation.However, given lack of improvement high-dose steroids (methylprednisolone 1000 mg daily for 3 days) were initiated and yielded significant improvement. Indeed, the patient was weaned to 2 LPM on the second day of high-dose steroids without concomitant psychosis. Subsequently, the patient was discharged on tapered steroid regimen and trimethoprim-sulfamethoxazole for PCP prophylaxis due to immunocompromised status. Two weeks after discharge, the patient was comfortable on room air and continuing the pulmonary rehabilitation.
Discussion: ILD, a common extra-articular manifestation of RA, is associated with higher mortality in the first 90 days post-acute exacerbation (AE) due to the independent predictors, such as low FVC at baseline and low PaO2 to FiO2 ratio [1]. The ILD risk factors of this patient were older age and seropositivity for rheumatoid factor. Radiological evidence of RA-ILD is frequently low-sensitive with nonspecific findings, including bibasilar ground-glass opacities, honeycombing and reticular or nodular involvement similar to our patient’s CT [2]. Despite the lack of consensus of RA-ILD management, the AE regimen typically includes high-dose corticosteroids and antibiotics [3].It is unclear if the AE is an overdriven autoimmune process, an independent inflammatory process, or both. As a lifetime non-smoker and no other risk factors, ILD is the most consistent attributable cause for this patient’s presentation. The interesting complexity of transiently reduced LVEF can likely be attributed to the severity of AE of ILD as LVEF improved after AE resolved.
Conclusions: RA-ILD may be a complex interaction between autoimmune and independent inflammatory processes. Evidence for RA-ILD long-term management is inconsistent, but the consensus is to treat the underlying RA, although its role in preventing AE of ILD is unclear. Further research can help distinguish the pathology and clarify the treatment of RA-ILD AE.