<unk>  <unk>  is an inflammatory condition characterized by high spiking fever, rash, sore throat, and joint pain. In 1896, an English doctor named George Frederick<unk> , described the condition in children and the<unk>  is named after him. In 1971, EG Bywaters described the term<unk><unk> 's<unk> " which was later used for<unk> s who had a condition similar to systemic<unk>  JRA.<unk>  <unk>  may be classified based on the predominant clinical picture with which the patient presents into systemic sub-type and chronic arthritis sub-type. The sub-types differ based on the cytokine profile, clinical course and response to treatment. It  is an autoimmune inflammatory arthritis that typically affects adolescents and<unk> s ranging from age 16-40 years. Major etiological mechanisms behind cause a dysfunction of the innate and cellular immunity (limited) leading to activation of effector cells of the<unk> . Although the pathogenesis of<unk>  is largely knwon to be idiopathic. Triggers of<unk>  lead to activation of toll-like receptors (TLR) and activation of immune system. Pathogen-associated molecular patterns (PAMPs) and danger-associated molecular patterns (DAMPs) play an important role in the etiopathogenesis of<unk> . They lead to release of various cytokines in the body such as interleukin-1 beta (IL-1), interleukin-6 (IL-6), interleukin-17, interleukin-18, interferon-alpha (IFN-alpha) and tumor necrosis factor (TNF-alpha). On gross examination, the involved joints may exhibit soft tissue swelling, cartilage loss, jointerosions and carpal ankylosis (late during the<unk>  process). On microscopic examination of the joint fluid, typical features associated with inflammatory joint<unk>  may be observed. There is a dearth of data regarding the incidence and prevalence of<unk>  <unk> . In the Japanese and the European populations, the reported prevalence rates range from 10 to 340 cases per 100,000 individuals. The<unk>  occurs worldwide and usually affects young<unk> s (age 16-35 years). If left untreated,<unk>  <unk>  follows a relapsing and remitting course. Initial presentation of<unk>  may be between 16 to 35 years of age. Symptoms usually evolve over weeks to months. Life-threatening complications known to be associated with<unk>  include, macrophage activating syndrome (MAS), disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura (TTP), diffuse alveolar hemorrhage, pulmonary arterial hypertension (PAH), pericardial tamponade and myocarditis. The prognosis of<unk>  depends upon the clinical course of the<unk> . The chronic articular form of the<unk>  is associated with worse prognosis. The diagnosis of<unk>  <unk>  is made clinically along with supporting laboratory abnormalities and exclusion of other rheumatologic disorders, malignancy and infections. Three diagnostic criteria have been established in order to aid in the diagnosis of<unk> . Yamaguchi criteria is widely adopted. A patient suffering from<unk>  may present with a fever greater than equal to 39 degrees celcius (102.2 degrees Fahrenheit) for greater than equal to 1 week along with associated arthralgia/arthritis for greater than equal to 2 weeks. There may be a maculopapular non-pruritic rash on the trunk or limbs. Patients may also complain of sore throat and lymphadenopathy. The symptoms of<unk>  evolve over a period of weeks. On x-ray of the affected joints, soft tissue swelling and joint effusions may be seen during early course of the<unk> . Intercarpal and carpometacarpal joint space narrowing (JSN) and ankylosis may be observed. MRI is more sensitive than x-ray in detecting bony erosions. It can detect synovitis, inflammation of the lining of the joints and tendon abnormalities. Medical therapy in<unk>  <unk>  is guided by<unk>  activity and severity. Corticosteroids are the first line therapy;<unk>  modifying anti-rheumatic drugs (DMARDs) as monotherapy or in combination with other agents may be used in refractory or complicated cases.
Adult-onset Still's disease
Boutonneuse fever
Relapsing fever
Brazilian purpuric fever
Paratyphoid fever
Lyme disease
Cotton fever
Periodic fever syndrome