Poster Session C
Periodic fever syndromes, autoinflammatory diseases, Still’s disease and MAS/HLH
Aleeza Qamar, DO
LSU Health-Shreveport
Shreveport, Louisiana, United States
No financial relationships with ineligible companies to disclose
The mean age of all patients (n=48) was 55.5 years (SD=11.5). Among all patients, 66.7% were female. Most patients were Black (93.8%). Approximately 81.3% of patients had biopsy-proven sarcoidosis. Lymphadenopathy was present in 85.4% of patients overall, with interstitial lung disease (ILD) and ground glass opacities (GGOs) present in 23.9% of patients overall. Fibrosis was present in 14.6% of patients overall, and abnormal PFT results were found in 37.5% of patients.
Other organ involvement was observed in 45.8% of patients overall. CT improvement was observed in 45.8% of patients overall, with similar percentages in both the TNF alpha (44.4%) and non-TNF alpha (46.7%) groups. There was no statistically significant difference between the groups (p=0.632).
Various treatments were administered, including Mycophenolate, Prednisone, Methotrexate, Azathioprine, HCQ (Hydroxychloroquine), Rituxan, and Arava. The proportions of patients receiving these treatments were generally comparable between the TNF alpha and non-TNF alpha groups, with no statistically significant differences observed for most treatments.
Conclusion:
In conclusion, this retrospective study offers insights into pulmonary sarcoidosis management at our center, highlighting common manifestations like lymphadenopathy, interstitial lung disease, and ground glass opacities. Extrapulmonary organ involvement was prevalent, underscoring the systemic nature of the disease.
Limitations include the small sample size and potential benefits of incorporating detailed measures like serial PFT findings to enhance outcomes. Treatment typically begins with glucocorticoids, followed by csDMARDs, and anti-TNFs for refractory cases or to reduce glucocorticoid use. Mycophenolate, Prednisone, and Methotrexate were commonly prescribed, often with TNF-alpha inhibitors, yet treatment outcomes, including CT scan improvements, did not significantly differ between TNF alpha inhibitors and other therapy groups.
A. Qamar: None; U. Agrawal: None; K. Sachdeva: None; Z. Hassan: None; A. Zamora: None; S. Nida: None; S. Umer: None; S. Hayat: None; K. Muzaffar: None.