Pediatric Rheumatology Online Journal → June 2003 → Systemic Onset Arthritis → Abstract #17

PRURITIC, LINEAR, URTICARIAL RASH IN 5 ADOLESCENTS WITH SPIKING FEVER AND SYSTEMIC ILLNESS: IS THIS SYSTEMIC ONSET JUVENILE IDIOPATHIC ARTHRITIS (SOJIA)?

L. B. Tucker,¹ D. A. Cabral,¹ J. S. Prendiville.²

¹Rheumatology, BC Childrens Hospital, Vancouver, BC, Canada; ²Dermatology, BC Childrens Hospital, Vancouver, BC, Canada

ILAR classification criteria for SOJIA require the presence of a daily spiking fever 39.5C for at least two weeks along with arthritis of one or more joints, typical rash, lymphadenopathy, hepatosplenomegaly, and serositis. Objective We describe 5 adolescent patients(ages 12-15 y) whose acute febrile systemic illness strongly resembled SOJIA, without sustained arthritis. Observations Clinical features were:daily high spiking fevers(5/5), arthralgia/myalgia(5/5), and hepatosplenomegaly(3/5). The rash was typical in appearance to SOJIA rash; it fluctuated with fever spikes, with erythematous maculopapular lesions and areas of linearity. However, the rash was intensely pruritic. Laboratory findings showed elevated ESR, anemia, leukocytosis, and thrombocytosis; 4/5 had elevated Factor 8 related antigen, and ferritin was elevated in two patients in whom it was measured. Extensive evaluation for infection was negative. Bone marrow examination showed mild hemophagocytosis in 3 patients; one patient developed thrombotic thrombocytopenic purpura. Three patients were treated with prednisone, with dramatic improvement. Resolution of symptoms was seen in one patient who was treated only with naproxen. One patient who was treated with naproxen died one day after being discharged from a two week hospital admission. She developed a severe generalized tonic-clonic seizure at home, with subsequent cardiac arrest. Post-mortem exam showed prominent lymphoid aggregates in the spleen and lymph nodes, skin vasculitis, and vasculopathy and microthrombi in multiple organs. No patient has developed arthritis after disease onset(F/U 1-8 y). One patient had two recurrences of rash and elevated acute phase reactants; with resolution with prednisone.Conclusion These five patients have had a multisystem inflammatory illness that closely resembles systemic onset JIA, but without chronic arthritis. Do these patients have a new disease, or do they have systemic JIA without arthritis?