Anti-CD-20 Therapy in Refractory Adult Still’s Disease

Reem Hamdy A Mohammed

doi:10.4236/ojra.2012.22002

Abstract

Adult Still’s disease is a relatively rare form of rheumatoid arthritis with systemic inflammatory features. The prevalence is around 1.5 cases per 100,000 - 1000,000. In the current case we display a 30-year-old male patient with refractory adult still’s disease who suffered recurrent attacks of fever 39.5°C, arthritis in proximal interphalangeal joints (PIPs), wrists, tempromandibular joints (TMJs), knees and ankles, stitching chest pain, dyspnea, erythematous rash over the trunk, sore throat, weight loss (15 Kilograms in 4 months). The patients’ disease remained uncontrolled despite of synthetic disease modifying anti-rheumatic drugs and repeated intramuscular corticosteroid injections. Laboratory workup revealed erythrocyte sedimentation rate (ESR) of 95, C-reactive protein (CRP) of 100 mg/L, hemoglobin 10.5 gm%, leukocytosis 12,000/microlitre, mild elevation of liver function tests and dyslipidemia. Serology revealed negative rheumatoid factor, anti-nuclear antibody titre of 1:80, elevated serum ferritin 4000 micrograms/litre. The patient was started on rituximab (375 mg/m2), prednisolone 20 mg/day and selective Cox-2 inhibitor. Follow up for over three months following the completion of his pulse therapy, revealed no relapse of fever or fatigue, with morning stiffness of 5 - 10 minutes, VAS of 3, DAS28 of 3.8, HAQDI of 0.62, ESR 23, CRP 4.9, Hb 12.5 gm%, leucocytic count 9000/microlitre, the dose of prednisolone was successfully reduced to a dose of 5 mg/day orally. Conclusion: Anti-CD20 therapy successfully controlled systemic and articular refractory disease with sustained efficacy over a follow up period of up to 24 weeks.

Highlights

Adult onset Still’s Disease (AoSD) is a relatively rare form of chronic inflammatory arthritis, that usually presents by high spiking fever, arthritis, myalgias, salmon pink skin rash, amongst other systemic inflammatory features
Follow up for over three months following the completion of his pulse therapy, revealed no relapse of fever or fatigue, with morning stiffness of 5 - 10 minutes, visual analogue scale for pain (VAS) of 3, DAS28 of 3.8, HAQDI of 0.62, erythrocyte sedimentation rate (ESR) 23, C-reactive protein (CRP) 4.9, Hb 12.5 gm%, leucocytic count 9000/microlitre, and the dose of prednisolone was successfully reduced to a dose of 5 mg/day orally
Three months following the completion of his pulse therapy, the patient reported disappearance of fever, fatigue, morning stiffness was 5 - 10 minutes, the patient was able to pray and to perform his daily activities without difficulty, with VAS of 3, DAS28 3.8, HAQDI of 0.62, ESR 23, CRP 4.9, Hb 12.5 gm%, leucocytic count 9000/ microlitre, the dose of prednisolone was successfully reduced to a dose of 5 mg/day orally without reported relapse of pain or fever

Summary

Introduction

Adult onset Still’s Disease (AoSD) is a relatively rare form of chronic inflammatory arthritis, that usually presents by high spiking fever, arthritis, myalgias, salmon pink skin rash, amongst other systemic inflammatory features. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) are classically negative. The Yamaguchi criteria are classified into four major criteria including: 1) Fever of at least 39 c for at least one week, 2) Arthralgias or arthritis for at least two weeks, 3) Non-pruritic salmon colored rash usually on the trunk and the extremities at the time of fever, 4) Leukocytosis 10,000/microlitre or greater, with granulocyte predominance and five minor criteria including: 1) Sore throat, 2) Lymphadenopathy, 3) Hepatomegaly or spelnomegally, 4) Abnormal liver function tests, 5) Negative tests for anti-nuclear antibody and rheumatoid factor. For establishing the diagnosis of AoSD the patient must satisfy 5 criteria with at least two major criteria [5]

Case Report

Management

Discussion

Conclusion