Case report- Adult StillsDisease - An elusive diagnosis Dr Ayunga - - PowerPoint PPT Presentation

Case report-Adult Still’sDisease- An elusive diagnosis

Dr Ayunga Physician Garissa

• A 23 year old gentleman of African descent
• presents with a skin rash,joint pains and fever for a

duration of 3 weeks.

• The fever was high grade but the patient was not

sick looking.

• Temperature ranged from 38-40 degrees celsius.
• He had generalized joint pains but had no joint

effusions.

• The patient had evanescent salmon-colored

nonpruritic rash that was mainly on the chest and abdomen.

• A full hemogram revealed a white blood cell

count of 27x109 with 90% being neutrophils.

• A blood culture was negative .
• A bone marrow aspirate and peripheral blood

film excluded leukemia as it reported a normal marrow.

• Anti-Cyclic citrullinated antibodies (Anti-ccp)

were negative at 2.0 EU/ml (0-10).

Treatments

• Patient was initially treated for septicemia with strong

antibiotics despite a negative blood culture but without improvement.

• Patient was then sent for a consultation in which Adult

Still’s disease was suspected because of the spiking fevers, elevated WBCC ,the rash on the trunk and the arthritis.

• The patient was put on high-dose aspirin at 900mg 1 g

tid,hydroxychloquine 200mg bd and prednisone at 15 mg tid.

• The patient responded quite well but the condition has

been recurrent on stopping the treatment.

Discussion

• ADULT STILL'S DISEASE
• Still's disease is considered a variant of

rheumatoid arthritis in which high spiking fevers are much more prominent, especially at the

• utset, than arthritis.
• This syndrome also occurs in adults.
• Most adults are in their 20s or 30s; onset after

age 60 is rare.

• The fever is dramatic, often spiking to 40°C,

associated with sweats and chills, and then plunging to several degrees below normal.

• Many patients initially complain of sore throat.
• An evanescent salmon-colored nonpruritic rash,

chiefly on the chest and abdomen, is a characteristic feature.

• However, the rash can easily be missed since it
• ften appears only with the fever spike.
• Many patients also have lymphadenopathy and

pericardial effusions.

• Joint symptoms are mild or absent in the beginning,

but a destructive arthritis, especially of the wrists, may develop months later.

• Anemia and leukocytosis, with WBCC

exceeding 40,000/mcL, are the rule.

• Ferritin levels are often exceptionally high in

adult Still's disease—for reasons that are not clear.

• The diagnosis requires exclusion of other

causes of fever.

• The diagnosis of adult Still's disease is strongly

suggested by the fever pattern, sore throat, and the classic rash.

• 50% of the pts respond to high-dose

aspirin or other NSAIDs

• 50% require prednisone, sometimes in

doses greater than 60 mg/d.

• TNF inhibitors may be helpful for some

pts with refractory adult Still's disease, but most patients treated with these agents achieve only partial remissions.

• About one-third of pts have recurrent

episodes.