Systemic juvenile idiopathic arthritis=still’s disease
onset at any age, M=F
•once or twice daily fever spikes(>38.5oC)≥ 2d/wk with temperaturere turning below baseline; children
usually acutely unwell during fever episodes
• extra-articular features: erythematous “salmon-coloured” maculopapular rash, lymphadenopathy,
☆hepatosplenomegaly, leukocytosis, thrombocytosis, anemia, serositis
•arthritis may occur weeks to months later rheumatology国試過去3回 HirotoShishido
first order test for systemic lupus erythematosusantinuclear antibody (ANA) titer is very sensitive(95-100%) and should be obtained as the initial ★first-order test
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if positive, more specific confirmatory tests such as anti-double-stranded DNA and anti-Sm (Smith) antibodies can be obtained
treatment for organ threatning disease in SLEIV cyclophosphamide for serious organ involvement (e.g. cerebritis or lupus nephritis) for clinical features of lupus nephritis rheumatology過去3回国試 HirotoShishido
manifestation of dermatomyositisstriated muscles and skin are affected
・Proximal muscle weakness affecting both sides of the body (progresses within weeks to months)
・Respiratory failure
・Myocarditis
・Esophageal disease(☆perifascicular atrophy of muscle fibers in the proximal esophageal muscularis propria)
・ovarian cancer
dermatologic
・☆heliotrope rash(pic), gottron papules
・Mechanic's hands: thickening and hyperpigmentation of the skin of the tips and sides of the fingers, resulting in irregular, dirty-appearing patc... musculoskeletalrheumatologyUWorld HirotoShishido
what mediator is closely correlated with the severity of giant cell arteritis(temporal arteritis)the production of cytokines, particularily interleukin-6(IL-6)
short term treatment for rheumatoid arthritisNSAIDs, glucocorticoids can provide rapid symptom relief in the interim
disease-modifying agents (eg, methotrexate, sulfasalazine, hydroxychloroquine, minocycline), biologic agents (eg, TNF-α inhibitors)
→alleviate pain and inflammation and reduce long term joint destruction (the response typically takes weeks) rheumatologypharmacologyUWorld HirotoShishido
common cause of myopathy・glucocorticoid induced myopathy
(glucocorticoid favors catabolism and proteolysis to provide subtrates for gluconeogenesis and glycogenesis in the liver→ ☆increase liver enzymes)
progressive proximal muscle weakness without pain(-CK)
・polymyalgia rheumatica(-CK)
・inflammatory myopathy
・statin induced myopathy
・☆hypothyroid myopathy musculoskeletalrheumatologyUWorld HirotoShishido
histology of buerger disease
in what races does it have a propensity to occur?(thromboangiitis obliterans)
segmental vasculitis(= ☆inflammatory intraluminal thrombi) extending into contiguous veins and nerves
scleroderma
histopathological findings☆dermal layer expansion due to diffuse deposition of collagen (eosinophilic connective tissue), resulting in ☆atrophy of the intradermal adipose tissue and dermal appendages (ie, hairfollicles, glands). dermatologyrheumatologyUWorld HirotoShishido
where does it hurt in the patient with polymyalgia rheumaticapain and (morning)stiffness in proximal muscles(shoulders, hips) rheumatology HirotoShishido
what is rheumatoid factorIgM antibody that targets IgG Fc region(in 80%)
what disease is associated with anti-centromere antibodyNinety-six percent of patients with detected anticentromere antibody in the serum have CREST syndrome.
lung involvement in vasculitidesusually in small-vessel vasculitis
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GPA(granulomatosis with polyangiitis) (proteinase3(c) anti-neutrophil cytoplasmic antibody)
MPA (microscopic polyangiitis, myeloperoxydase (p) ANCA)
similar to GPA but without nasopharyngeal involvement