Adult Still disease

Inflammatory process with high fever that recurs daily, polyarthritis, rash, leukocytosis, and may be associated with lymphadenopathy, hepatosplenomegaly, and mild increase in liver function tests.

Fever typically quotidian and spiking, often in the evening.

Adult onset Still’s disease.

A form of Still’s disease, a rare systemic autoinflammatory disease characterized by the classic triad of persistent high spiking fevers, joint pain, and a distinctive salmon-colored bumpy rash.

Onset is associated with physical and emotional stress in year preceding manifestation of disease.

Etiology suggested to be due to immune dysregulation.

A diagnosis of exclusion.

A clinical diagnosis.

Incidence approximately 0.16 cases per 100,000 person-years.

Incidence slightly higher in women.

Mean age at presentation is 38 years.

Two thirds of patients older than 35 years of age (Kadar J).

Etiology unknown, but viral and bacterial pathogens suspected.

Arthritis prevalence estimated at 64-100% and can progress to joint deformities in patients with refractory or in adequately treated inflammatory joint disease.

Typically associated with normochromic anemia and thrombocytosis.

Pulmonary infiltrates and renal involvement may occur.

Rash is non pruritic, non tender, macular/papular, salmoned color on trunk and extremities.

The rash primarily affects the proximal limbs and trunk, sparing the limbs and face.

The rash can be misdiagnosed as a drug eruption, due to occasional mild pruritis and the tendency to occur during fever attacks.

Biopsy of rash reveals edema and perivascular inflammation.

Arthritis/arhrlagias involve knees, wrists, elbows, proximal interphalangeal joints and shoulders.

Synovial fluid analysis reveal inflammatory changes with a mean WBC count of 13,000mm3.

Mild increases of CPK and aldolase may occur, with myalgias, but muscle biopsy is normal.

Acute phase reactants ESR, CRP and f2242itin may be elevated.

Patients may present with pleuritis, pericarditis, pleural effusions, myocarditis, and transient pulmonary infiltrates.

There is often involvement of the eye, migratory myalgias, abdominal pain, and rash.

65% of patients have lymphadenopathy.

One third of patients have a sore throat and may be the initial presenting symptom.

Biopsy of lymph nodes reveal benign changes of polyclonal B cell hyperplasia.

70% of patients have mild elevations of liver function tests at some point during their illness.

Yamaguchi Diagnostic criteria (Yamaguchi A):


Arthritis or arthralgia 2 weeks or longer with swelling and limited movement

Fever 39C or greater, persistent or intermittent for 1 week or longer.

Typical evanescnt, salmon colored, macular/papular on trunk or extremities, associated with fever.

Leukocytosis 10,000mm3 or higher and 80% neutrophils.


Sore throat

Lymphadenopathy and or splenomegaly

Elevated liver functions

Negative rheumatoid factor and ANA tests


Infections, malignancies and rheumatic diseases.

Diagnosis requires 5 criteria and at least 2 of them must be major in nature.

Yamaguchi criteria has a relatively high sensitivity and specificity , at 96.5% and 92.1% respectively.

Fever usually daily or twice daily and usually in the evening.

Wide fever fluctuations occur and in 20% of patients temperature does not resolve between spikes.

Three distinct patterns of disease: self-limited or monocyclic, intermittent or polycyclic and chronic articular.

Monocyclic disease has a systemic presentation that resolves completely within one year.

Self-limiting course in one third of patients and can take up to one year to resolve.

One third of patients have persistence of active disease with destructive arthritis.

Polcyclic is a relapsing form, with complete remission in between episodes.

Chronic articular adult onset Still’s disease is often severe with poor prognosis leading to joint distruction.

Macrophage activation syndrome, is a recognized complication of disease with an excess of activation of the reticuloendothelial system leading to macrophage activation and the destruction of hematopoetic cells.

Macrophage activation syndrome occurs in 10-17% of cases, with a mortality rate of 10-22%.

Non steroid anti-inflammatory drugs and corticosteroids are the primary treatments to manage fever, inflammation, arthritis.

Second line treatments include tumor necrosis factor inhibitors, infliximab and etanercept.

Methotrexate may be used if steroids are contraindicated.

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