Adult onset Still’s disease (AOSD) is an auto-inflammatory disorder of unknown etiology & can manifest in multiple forms like pyrexia of unknown origin, serositis, aseptic meningitis, myocarditis, hemophagocytic syndrome and septic arthritis.
In the JAPI journal researchers report a case of an asplenic patient who presented with fever and polyarthralgia in whom AOSD was diagnosed. This is the first case report of AOSD in an asplenic patient from India.
A case of post-splenectomised man, presented with high grade fever, joint pain and body ache. Since Overwhelming Post Splenectomy Infection (OPSI) was the initial probable diagnosis, empirical antibiotic therapy was initiated. Evaluation to find a septic focus, autoimmune diseases ad malignancies was carried out which showed negative results. Since alternative diagnoses were excluded and the Yamaguchi’s criteria for AOSD was fulfilled, the patient was treated with IV steroids which resulted in rapid resolution of his symptoms. AOSD with asplenia is a unique condition because immunosuppressive therapy for AOSD may increase the risk of OPSI in such a patient.
The patient was given three daily pulses of 500mg of intravenous methylprednisolone which resulted in rapid resolution of his symptoms. After a week of steroid therapy, he was discharged on 1 mg/kg of oral prednisolone and 15 mg/ week of methotrexate. The patient has been doing well on follow-up. Steroids were tapered to 5 mg/day of prednisolone. Methotrexate is being continued in a 15 mg weekly dose.
The presentation of fever with asplenia made doctors focus on OPSI by an encapsulated organism. But when the patient did not improve with initial management, it made them look for alternative diagnoses thus leading to the rare diagnosis of AOSD. Keeping an open mind while evaluating the patient can prevent an unusual disease from being misdiagnosed.
Source: JAPI, India