Non-purulent pharyngitis: adult still’s disease

ABSTRACT

 Adult Still’s disease is rare and may present as pyrexia of unknown origin. Due to lack of expertise, diagnosis may be delayed inadvertently. The patient usually presents with spiked fever, polyarthralgia or arthritis, evanescent skin rash, non-purulent pharyngitis, lymphadenopathy and hepatosplenomegaly. Leukocytosis, predominantly of neutrophils, elevated erythrocyte sedimentation rate and C-reactive protein without obvious infection are the hallmarks of the disease. Delay in diagnosis may expose the patient to the side effects of antibiotics as they are repeatedly prescribed in view of elevated leukocytes. The majority of patients report pain in the throat without evidence of infection. This was an important clue to our diagnosis of this patient. Grossly elevated serum ferritin is diagnostic of adult onset still's disease. As the white cell counts are grossly elevated, a bone marrow examination to rule out hematological malignancy may be mandatory. Serum ferritin value has prognostic value too. Minor illness may respond to non-steroidal anti-inflammatory drugs (NSAIDs), but steroids are the mainstay of the treatment.  Methotrexate is of additional value for those presenting predominantly with arthritis. Anakinra, Infliximab and Tocilizumab are other options. Those patients presenting with severe disease and organ involvement require high dose intravenous steroids followed by high dose oral steroids.