Abstract
BACKGROUND: Acute interstitial pneumonia (AIP), also known as Hamman-Rich syndrome, is a rapidly progressive interstitial lung disease. In addition to being challenging to diagnose, AIP is also difficult to treat. The mortality rate of AIP is greater than 70% due to the disease's rapid progression. Furthermore, survivors are likely to develop chronic interstitial lung disease as a sequela. Treatment primarily focuses on supportive care, which consists of oxygenation through mechanical ventilation, administration of broad-spectrum antibiotics, and the use of corticosteroids. Although the use of steroids as empiric treatment is controversial and results on its mortality benefit are variable, some studies have shown high-dose pulse steroid therapy to be associated with better health outcomes. This review aimed to evaluate cases of AIP to better understand the role of corticosteroids in the management plan of these cases. METHODS: A systematic review was conducted using the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) 2020 guidelines. Outcomes of interest included patient age, sex, autoimmune condition, corticosteroid use, survival or expiration of patients, and time from hospitalization to expiration. RESULTS: Initial querying of the five databases yielded 376 articles. Following a thorough review, only 30 articles remained, comprising 42 patient cases. Of these cases, 62% of the patients survived, 36% expired, and 2% were unknown. The average stay from hospitalization to death was 20.2 days, and corticosteroid pulse doses were used as a first- or second-line treatment in 31% of patients. CONCLUSION: The limitations of the evidence used in this study highlight the need for a greater output of higher-level evidence in the form of controlled trials and retrospective studies to help further elucidate the proper role and dosage of corticosteroids in the management plan of AIP with the ultimate goal of enhancing clinical decision-making and patient care. The findings of this systematic review, primarily based on observational data from case reports, highlight the critical need for treatment guidelines for this condition. The compilations of these cases also illustrated the diverse strategies employed by clinicians globally to save patients afflicted by this condition. While specific recommendations cannot be made based solely on these results, we anticipate that this comprehensive overview of varied clinical approaches from around the world will serve as a valuable resource for healthcare providers navigating the complexities of managing this condition.