Académique Documents
Professionnel Documents
Culture Documents
Vinod Chandran et al
However, the fever can have more than one spike and may be
continuous. Characteristically, the patient looks well when he
is afebrile. Around 90% of patients also have sore throat due
to non-exudative pharyngitis. Most patients also develop
weight loss of more than 10%.
The typical Stills rash is an evanescent, salmon
pink macular or maculopapular rash, seen along with the spike
of fever on the trunk and proximal extremities. Even though
>90% patients in series reported from the West 2,3 have this
characteristic rash, this has been less reported in studies from
India4,5 probably due to the difficulty in identifying the rash in
people with dark skin. The rash can be induced by minor
trauma (Koebner phenomenon). Dermographism has also been
described.
Arthritis occurs in about 95%, 2,3 and flare up of
joint symptoms occurs during the febrile spike. As the
duration of the disease increases the arthritis becomes more
prominent and the fever decreases in intensity. The most
common joints involved are the knees, followed by the wrists,
ankles, elbows, metacarpophalangeal, metatarsophalangeal,
and distal interphalangeal joints, hips, neck and
temperomandibular joint and interphalangeal joints of the feet.
Polyarthritis is most common. However, a few patients have
oligoarthritis and rarely a single joint is involved. 5% of
patients do not have arthritis. 2 About 18% have distal
interphalangeal joint involvement.2 Radiographs of the affected
joints in early disease show juxta-articular osteoporosis and
soft tissue swelling. Later in established arthritis, erosions and
joint space narrowing might be detected. A selective
non-erosive narrowing of the carpometacarpal and
intercarpal joint spaces, later leading to a intercarpal bony
fusion is more specific for AoSD as compared to more
radiocarpal involvement in rheumatoid arthritis.9 Ankylosis of
the apophyseal joints of the cervical spine occurs uncommonly.
Destructive arthritis of the hips occurs in 5-33% of patients.2,3
Lymphadenopathy, splenomegaly and hepatomegaly are detected in 63, 52 and 42% of patients, respectively2,3.
Cervical lymph nodes are most commonly affected. About a
third of patients have pleural effusion, and 27% of patients
have pneumonitis affecting the upper or lower lobes. Rarely,
patients develop acute respiratory distress syndrome and
interstitial lung disease. Pericarditis is seen in about a third of
patients rarely leading on to tamponade. Myocarditis has also
been reported. Close to 50% patients have abdominal pain
and can occasionally simulate an acute surgical abdomen2,3.
Rare manifestations like secondary Sjogrens
syndrome, aseptic meningitis, cranial nerve palsies,
peripheral neuropathy, retinal lesions, iritis, panophthalmitis,
interstitial nephritis, glomerulonephritis and thrombotic
thrombocytopenic purpura have also been described.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
21