Treatment of systemic lupus erythemus overlap syndrome with autoimmune hepatitis using a combination of glucocorticoids and immunosuppressive agents: Case report.

Rationale: Systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) are distinct autoimmune disorders whose concurrent presentation (overlap syndrome) poses significant diagnostic and therapeutic challenges. While SLE-associated liver involvement is recognized, true SLE-AIH overlap is rare and lacks established management guidelines. This report details the successful management of such a case to contribute insights into this complex clinical scenario.

Patient Concerns: A 35-year-old male presented with a 1-year history of intermittent facial and body rashes accompanied by significant weight loss (approximately 10 kg). Over the preceding half-month, he developed signs of liver injury, including fatigue, cold intolerance, loss of appetite, and generalized weakness.

Diagnoses: Comprehensive diagnostic evaluation, cutaneous manifestations: facial and generalized skin rash, hematologic abnormalities: cytopenia, including serological testing (positive antinuclear antibody [1:1000], anti-SS-A antibodies, elevated immunoglobulin G, rheumatoid factor, erythrocyte sedimentation rate, C-reactive protein, decreased complement C3 and C4 levels), liver function tests (persistently elevated alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, alkaline phosphatase, globulin), and imaging (uneven liver texture/density on ultrasound/computed tomography), confirmed a diagnosis of SLE complicated by AIH. The diagnosis of SLE meets both the 1997 American College of Rheumatology classification criteria and the 2012 Systemic Lupus International Collaborating Clinics revised criteria. AIH diagnosis was supported by both the 1990 (score=16) and 2008 (score=7) International Autoimmune Hepatitis Group (IAIHG) scoring systems. Infectious and other common causes of liver injury were excluded.

Interventions: Initial management focused on controlling liver injury using intravenous and oral hepatoprotective agents (inosine, bicyclol, silibinin, compound glycyrrhizin, magnesium isoglycyrrhizinate). High-dose intravenous glucocorticoids (dexamethasone 5-10 mg daily) were initiated concurrently to suppress autoimmune activity. Upon stabilization of liver function, treatment transitioned to oral prednisone (30 mg daily) combined with the immunosuppressant leflunomide (10 mg daily). Glucocorticoids were subsequently tapered strictly according to plan, while leflunomide monotherapy was maintained long-term. Adjunctive therapies included calcium and gastric protection.

Outcomes: Liver function tests normalized following the combined hepatoprotective and glucocorticoid therapy. The introduction and maintenance of leflunomide, alongside glucocorticoid tapering, effectively controlled SLE disease activity. At a 2-year follow-up, the patient remained clinically stable with normalized liver function, no recurrence of severe symptoms, and a good quality of life.

Lessons: This case highlights the efficacy of a sequential treatment approach combining glucocorticoids and immunosuppressants (specifically leflunomide) for managing SLE-AIH overlap syndrome. Early diagnosis using established criteria is crucial. Multidisciplinary collaboration is essential. Long-term leflunomide monotherapy demonstrated sustained remission in this patient, although vigilance for adverse effects (e.g., hepatotoxicity) and disease flare is necessary. The absence of specific guidelines for this overlap syndrome underscores the need for individualized treatment strategies based on SLE and AIH management principles.