| What is adult Still's disease? | Systemic inflammatory disorder of unknown etiology, clinical triad (daily spiking high fevers, evanescent rash, arthritis)
Biological tiad (hyperfirritinemia, hyperleukocytosis with neutrophilia, abnormal LFT)
Is is a rare disorder, equal distribution between sexes, between 16-35 years old, <16 years have systemic-onset juvenile idiopathic arthritis (SOJIA) |
| How is ASD pathogenesis? | Genetics (unknown, HLA?)
Environment (infections like rubella, echovirus 7, mumps, EBV, CMV, parainfluenza, parvovirus B19, Coxsackie, hepB/C, HSV, microbial like M.pneumonia, C.pneumonia, Brucella, Borriella, Yersinia)
Cytokines (IL1, IL6, IL18, TNF-a and IFNy) |
| What are clinical manifestations of ASD? | Fever (96%, sudden onset spiking high fever, peak once daily late afternoon/early evening, exceeds 39 degrees, lasts under 4 hours, returns normal 80% of time without antipyretic)
Rash (73%, evanescent, salmon-pink, macular/maculopapular, proximal limbs and trunk, emerges with fever in evening, koebner/mild pruritis)
Musculoskeletal (arthritis 64-100%, may be mild, oligoarticular, transient, evolves over several months to more severe destructive symmetrical and polyarticular form, knees, wrist and ankles, elbows..., progressive changes in wrist 6 months after onset, develop ankylosis 1.5-3 years later, myalgia and myositis)
Sore throat (69%, typical sign, common in 1st month subsequent flares)
Liver disease (50-70%, hepatomegaly and LFT abnormal, fatal fulminant)
Lymphadenopathy and Splenomegaly (50%, tednerness, biopsty polyclonal B cells hyperplasia)
Cardiopulmonary disease (30-40%, pleuritis, pericarditis, ARDS, Tamponade)
Hemato (pancytopenia due to hematophagic syndrome) |
| What are labs of Still's disease? | High ESR/CRP, leukocytosis and neutrophilia, anemia of chronic disease, pancytopenia, DIC, high LFTs, serum ferritin high (under influence of IL1 and IL6 acute phase reactant correlates with disease activity, very high 3000-30000, >10000 hematophagic syndrome)
Glycosylated ferritin (drops to 20-50% in inflammatory disease, due to saturation of glycosylation mechanism, in ASD drops under 20%) |
| What are radio findings in still's disease? | No sepcifc finding, on X ray normal to erosion to ankylosis. |
| What are classification criteria of Still's disease? | dx need 5 criteria or more with at least 2 major criteria
Major (Fever intermittent >39C for 1 week or longer, arthralgia/arhtritis for 2 weeks or more, typical rash, leukocytosis 80% granulocytes)
Minor (sore throat, lymphadenopathy, hepatomegaly, abnormal LFT, - ANA/RA)
Exclusion criteria (Infections, malignancies, other rheumatic disorders) |
| How is the course of ASD? | ▪ Self-limiting or monophasic pattern (single disease episode, achieve complete remission within one year)
▪ Intermittent or polycyclic systemic pattern (recurrent disease flares with systemic symptoms +/- articular symptoms, complete remission of symptoms between flares)
▪ Chronic articular pattern (persistent active disease with predominantly articular symptoms, Patients may have severe, destructive arthritis) |
| How is the prognosis of ASD? | articular symptoms poor prognosis due to disability more than systemic symptoms, could be life threat if hematophagic syndrome, , ARDS, DIC.
Functional status of ASD is good prognosis, joint arthroplasty improved pt. |
