Dr Anakha V Ajay* Dr C Jayakumar* (Paediatrics )Dr Suma Balan** Paed Rheumatology)AIMS, Kochi
One year six month old female child presented with high grade fever associated with rash at the spike of fever which used to disappear each and every time fever subsides for the last 20 days Initially the fever was quotidian with evanescent rash but later fever was persistant with fixed rash. Outside health care facility managed as MISC as child had high covid IgG antibody titre and raised inflammatory markers.She was managed with pulse steroid and enoxaparin. Poor clinical response lead to referral. All history prior to admission like development Diet,immunisation were normal
Family history was non contributory
At admission febrile irritable child had erythematous rashes present all over the body. Other PICCLE findings were absent Soft Liver noted 1cm below the costal margin. Other systems were normal Differentials
Kawasaki disease,
MISC
Systemic onset juvenile idiopathic arthritis.
Drug rashes
SLE
LABS
TC-10.87K/uL N-46% L- 48%
CRP-33mg/L,
ESR-87mm/hr,
LDH-6606U/L ,
Ferritin-102243ng/ml
SGOT-2388IU/L,
SGPT-1131IU/L,
hypofibrinogemia(124mg/dl) triglycerides(252mg/dl).
ECHO done was normal with no evidence of coronary dilatation
.In view of clinical picture along with investigations , diagnosis of systemic onset juvenile idiopathic arthritis was made.
Child was started on pulse dose of steroids (IV Methylprednisolone) followed IV Anakinra.
As CMV PCR done showed high titres(3119) child was also started on IV Ganciclovor. Following this child had reduction in fever spikes and decreasing trend in inflammatory markers, hence anakinra and steroids were tapered and stopped. But child started getting further fever spikes, hence was initiated on second line immunosuppressive agent Cyclosporin..
CMV PCR was repeated and was decreased(<60), so oral valganciclovir was continued for 2 weeks.
Child improved with the treatment and was discharged on cyclosporin and oral steroids.
Systemic onset juvenile idiopathic arthritis(SoJIA) is a systemic inflammatory disease,and is a distinctive subtype of JIA.
It is characterised spiking fever, evanescent rash, hepatosplenomegaly, serositis, lymphadenopathy and arthritis. Key players of the innate immunity system are involved in the pathophysiology of SoJIA. Interleukins IL-6, IL-1, involved in innate immunity essentially contribute to the etiopathogenesis of SoJIA.
Laboratory investigations usually show elevated inflammatory markers.Myeloid reactive protein are present at very high circulating levels in SoJIA from other febrile conditions.Ferririn is often increased but high serum levels suggest macrophage activation syndrome.Treatment of mild to moderate cases with NSAIDS other than aspirin
in case of poor response
pulse steroids are considered.
Cases refractory to steroids, IL-1 and IL-6 inhibitors are used
Prognosis-There is no cure, but remission is possible.
Upto 30% of patients will still have active disease after 10 years and morbidity within this group is high.Complications of SoJIA include macrophage activation syndrome, limitations in functional outcome arthritis and long term damage from chronic inflammation.
Key take home message is early diagnosis and prompt, aggressive treatment to mitigate potential long term complications and improve patient outcomes.
