A Cohort Study of Liver Involvement in Patients With Adult-Onset Still's Disease: Prevalence, Characteristics and Impact on Prognosis

Abstract

Objective: Adult-onset Still's disease (AOSD) is a systemic disorder commonly accompanied by liver involvement. This study aims to illustrate the detailed information of liver abnormalities in patients with AOSD and evaluate the impact on the prognosis.Methods: A total number of 128 hospitalized patients, who met the Yamaguchi criteria of AOSD in the Department of Rheumatology and Immunology, Ruijin Hospital from July 2016 to August 2019 were consecutively enrolled and followed up. The demographic characteristics, clinical features, laboratory tests, treatments and prognosis were recorded. Correlations of liver function tests (LFTs) with disease activity and laboratory parameters were analyzed by the Spearman test. Risk factors of the refractory AOSD were evaluated by multivariate logistic regression analysis.Results: Liver involvement was presented in 104 (81.3%) patients with AOSD. We observed that 34 (32.7%) patients were with mild elevation, 32 (30.8%) patients were with moderate elevation, and 38 (36.5%) patients were with severe elevation. The majority of elevated ALT, AST and ALP decreased to normal within the range of 2 months, except for GGT. Furthermore, the LFTs were found significantly correlated with disease activity. Besides, we found patients with higher levels of LFTs tended to require more intensive treatments and suffered from poorer prognosis. Multivariate logistic regression analysis showed ALP ≥ 141 IU/L and GGT ≥ 132 IU/L are independent risk factors of refractory AOSD.Conclusion: Liver involvement is common in patients with AOSD, the levels of LFTs are associated with disease activity and related to the treatment strategies and prognosis.

Adult-onset Still’s disease with multiple lymphadenopathy: a case report and literature review

“…Secondary hemophagocytic lymphohistiocytosis, also known as macrophage activation syndrome, is the most severe complication associated with high mortality. Its common complications include coagulopathy with multiorgan involvement, including the heart, lung, liver, spleen, and other sites [ 17 , 18 ], and these patients often require more intensive treatment and have a worse prognosis. It has been shown that more than 20% of patients with AOSD experience recurrence and that patients with severe disease at the initial stage of the disease may be at an increased risk of recurrence, which requires intensive treatment and close follow-up [ 19 ].…”

Diagnosis and treatment of adult-onset Still’s disease: a concise summary of the German society of rheumatology S2 guideline

“…Therefore, these criteria can be employed to ascertain the clinical diagnosis of AOSD made by an expert [10,85,119] (see recommendation 2 in . Table 1 [44,55,100,174,177] (see recommendation 3 in . Table 1).…”

The Systemic Score May Identify Life‐Threatening Evolution in Still Disease: Data from the GIRRCS AOSD‐Study Group and the AIDA Network Still Disease Registry

“…38 Furthermore, serum prealbumin and albumin levels have been recently reported to be correlated with liver involvement and to negatively reflect the disease activity of patients, possibly as part of the acute phase response. 38 However, a more severe involvement with substantial cytolysis and hepatic necrosis may occur in connection with a multiorgan failure syndrome. 38,39 Although liver involvement is frequently observed in Still disease, it is quite heterogeneous.…”

“…38 However, a more severe involvement with substantial cytolysis and hepatic necrosis may occur in connection with a multiorgan failure syndrome. 38,39 Although liver involvement is frequently observed in Still disease, it is quite heterogeneous. Nonspecific histopathologic changes of liver involvement may be observed also featuring hemophagocytosis.…”

“…Nonspecific histopathologic changes of liver involvement may be observed also featuring hemophagocytosis. 8,38 Experimental models of hemophagocytic lymphohistiocytosis in association with the overproduction of cytokines have shown that hemophagocytosis may occur in the liver as well as in the bone marrow and lymph nodes. 40,41 Notably, patients with Still disease and liver involvement have been reported to have an almost sixfold higher risk of MAS than those without.…”