Hatem Eleishi, MD Rheumatologist STILL’S DISEASE.

Hatem Eleishi, MD

Rheumatologist

STILL’S DISEASE

Systemic onset juvenile rheumatoid arthritis (formerly called Still's disease)

is a subset of juvenile rheumatoid arthritis (JRA) that describes patients with:

Intermittent fever +/- rashArthritis

Children with this illness comprise between 10 and 15 percent of all cases of JRA.

By definition, systemic onset JRA does not occur after the age of 16 years.

Adult-onset Still's disease

Is the same disorder occurring in patients over the age of 16.

CLINICAL MANIFESTATIONS

Both sexes equallyAny age

Articular manifestations: Arthralgias: commonArthritis: may be delayedMicrognathia and cervical spine fusion common

Extra-articular manifestations: FeverMacular, salmon pink rash HepatomegalySplenomegalyLymphadenopathy Pericardial effusions

Disseminated intravascular coagulation (DIC) or Macrophage activation syndrome (MAS)

May occur within the first few days or weeks following: 1. Initiation of therapy with NSAIDs or gold salts 2. Viral and bacterial infections

Or may occurwithout any evident initiating event.

DIAGNOSIS:

A diagnosis of exclusion

Combination of intermittent daily fevers greater than 38.5°C and arthritis

For at least six weeks May be delayed

Hallmark

Laboratory findings:

Acute phase response

Neutrophilic leucocytosis: count in the 20,000 to 30,000/mm3 range

Marked reactive thrombocytosis

Anemia: commonly present and often profound

Elevated serum ferritin levels

Minor transaminitis

SOJRA may be the most difficult form of JRA to diagnose for the following reasons:

First:

Arthritis may not be evident early in the course of the disease.

Arthritis is important to make a

definite diagnosis

Second: Children often appear quite ill with:

High spiking feversRashes

Markedly elevated WBC countsAnemia

Hence, initially thought to have an infection

But, with no response to antibioticsAnd with >6weeks duration

**

An ill child with nausea,

fever, and an erythematous rash over the extremities

Viral exanthems in childhood

ParvovirusRubellaEB rarelyHBV

Third: Children may have:

FeversMarkedly elevated WBC counts

AnemiaLymphadenopathy

Hence, leukemia considered

Clues to differentiation include

Systemic onset JRA is not associated with:

ThrombocytopeniaLymphocytosisExtreme pain*

LN biopsy shows reactive hyperplasia

c

Five-year old boy with a 6 months’ history of fever (40oC), weight loss and polyarthritisOn examination: ill, pale and irritable, got apprehensive on any attempt to examine his joints. He had generalized lymphadenopathy. There was swellingand tenderness ofhis wrists, elbows, knees, anklesand feet joints.

His labs showed ESR 140Hb 2.3; MCV 56WBC 2.9Platelets 50Serum albumin 2.1ALT, AST: normalUrine: freeANA negative

Treatment: NSAIDs: Indomethacin

Corticosteroids: used judiciously to minimize toxicity (below 0.5 mg/kg per day of prednisone or its equivalent) and the duration of therapy should be less than six months

Methotrexate with a usual maximum oral dose of 15 mg per week.

Course and prognosis:

Highly variable, but in general:

The child who appears well after six months has a substantial probability of remaining well.

Poorer functional outcomes may occur in those with more than six months of fever, thrombocytosis, or corticosteroid therapy

In conclusion

In conclusion

SOJRA is:

Intermittent fever of at least 6 weeks’ duration

And arthritis

SOJRA is a diagnosis of exclusion

Treatment consists of corticosteroids that should be used judiciously and immunosuppressives as methotrexate

Prognosis can be predicted over the first 6 months of the course of the disease

Thank you