Systemic JRA: (Still's Disease)


- See: Juvenile Rheumatoid Arthritis

- Discussion:
    - characterized by high fever, rash, lymphadenopathy, and polyarthritis;
    - usually appears at 5-10 years of age;
    - least common type of JRA: < 20 % of patients.

- Clinical Presentation:
    - acute onset, often accompanied by a sore throat and leukocytosis, may suggest a bacterial infection;
    - fever:
         - temp > 40 deg is common (unlike rheumatic fever);
         - may be spiking & erratic; once or twice daily; must be > 39.3;
         - peaks usually at nite, improved by morning;
    - rash:
         - distinctive, pink rash blanches on compression;
         - macular or maculopapular w/ central clearing;
         - nonpuritic, pink or salmon colored & is common on trunk & axilla;
         - scratching may worsen rash (Kobner's phenomenon)
         - when pruritic, rash may be confused w/ hypersensitivity reaction;
         - may precede arthritis;
    - synovitis / joint pain:
         - in most pts arthritis is chronic & persistent but may be migratory at first;
         - usually more painful than Pauci or Poly JRA;
         - joint pain may be most intense as fever peaks;
    - neck stiffness is common (consider meningitis);
    - systemic manifestations:
         - pericarditis, myocarditis
         - lymphadenopathy (esp in axilla)
         - hepatosplenomegaly
         - abdominal pain.
         - growth retardation
         - vasculitis (including CNS)
         - asymptomatic iritis;

- Labs:
    - Synovial F. Exam
    - anemia & leukocytosis ( > 40,000 WBC) (thrombocytopenia & leukopenia are rarely found)
    - ESR is usually high, > 100 mm/hr.
    - ANA & RF are rarely positive.

- Prognosis: is variable
    - systemic manifestations may last for months;
    - large minority will develop severe arthritis:
    - hip disease is a major cause of late disability.

- Radiographs:
    - may see a developmental fusion of cervical vertebra that may be confused w/ fibrodysplasia ossificans



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Tuesday, August 21, 2012 4:24 pm