Adult-Onset Stills Disease – aka Adult Still’s, Wissler-Fanconi Syndrome

Acronym:
AOSD

SAID group:
Macrophage Activation Diseases

Gene:
Currently unknown. No genetic tests are available at this time.

Inheritance:
Currently unknown.

Ethnicity:
Rare. Affects all races. [44]

Frequency:
France: estimated that 0.16:100,000 people have AOSD. AOSD affects more women than men. [44]

Timing of symptoms:
High fevers > 39°C that last for <4 hours, recurring more than once a week, with a maculopapular rash and arthralgia. [44]

Age of onset:
First onset of symptoms occurs between 16-35 years of age. Affects all ages. [44]

Skin cutaneous:
Evanescent, salmon-pink, mildly pruritic maculopapular rash on the proximal limbs and trunk. [44]

Neurologic:
>95% have high, spiking fevers, fatigue and myalgia with flares. Other neurological symptoms are very rarely seen. [44]

Auditory:
Not noted. [44]

Ophthalamic:
Not noted. [44]

Cardiopulmonary:
<25% have pleuritis (pleurisy), pericarditis (a few with tamponade). Some myocarditis, pleural effusions, Adult Respiratory Distress Syndrome (ARDS). [44]

Abdominal:
50-75% with hepatomegaly, abnormal liver function tests (LFTs). 43% with splenomegaly. Some with hepatosplenomegaly. Renal disease is rare. [44]

Lymphatic:
Lymphadenopathy is common. Many with splenomegaly. [44]

Joints bones muscles cartilage:
Myalgias, arthralgias and/or arthritis are common. Wrist changes after 6 months. 41% develop intercarpal and carpometacarpal joint space narrowing a few years after onset of AOSD – 25% then develop pericapitate ankylosis. [44]

Vasculitis:
Not noted. [44]

Amyloidosis:
Very rare. [44]

Abnormal labs:
High: ESR, CRP, LFTs, Ferritin, IL-18. Low glycosylated ferritin. Leukocytosis, anemia common with flares. Prolonged PTT: risk for disseminated intravascular coagulation (DIC). [44]

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