MGR review 류마티스 내과 R4 최인아

[ 목차 ] Overlap syndrome (vs UCTD, MCTD) Polymyositis(PM) PM with Interstitial lung disease

[ 목차 ] Overlap syndrome (vs UCTD, MCTD) Polymyositis(PM) PM with Interstitial lung disease

Overlap syndrome vs UCTD / MCTD MCTD Overlap syndrome UCTD

여러 면역질환에서 공통적으로 나타나 특정 질환을 나타내기에는 불충분한 특징 Raynaud’s phenomenon, arthralgia, weak ANA 질병 Specific 한 특징들이 중복될 때 thickening of the skin proximal to fingers in scleroderma, articular erosions in RA

[ 목차 ] Overlap syndrome (vs UCTD, MCTD) Polymyositis(PM) PM with Interstitial lung disease

Causes of myopathies Idiopathic myopathy – immune mediated Acute alcoholic myopathy Infectious causes Endocrine causes Drug-induced or toxic Acute periodic paralysis –Trichinosis –Cysticercosis –Toxoplasmosis –HIV –Coxsackie A and B viruses –Influenza –Lyme disease –Staphylococcus aureus –Addison disease –Cushing disease –Hypothyroidism (CK may be mildly elevated) –Hyperthyroidism (CK may be normal) –Hyperparathyroidism –steroids –clofibrate, –statins –colchicine –amiodarone –penicillamine –drugs causing hypokalemia - hypokalemic, hyperkalemic, or normokalemic

a SLE, RA, Sjögren's syndrome, systemic sclerosis, MCTD. b Crohn's disease, vasculitis, sarcoidosis, primary biliary cirrhosis, adult celiac disease, chronic GVHD, discoid lupus, AS, Behçet's syndrome, etc c HIV and HTLV-I. d Drugs include penicillamine (dermatomyositis and polymyositis), zidovudine (polymyositis). e Parasites (protozoa, cestodes, nematodes), tropical and bacterial myositis (pyomyositis).

=Anti-JO 1 Anti synthetase syndrome

Mechanic's hands : anti-PM-Scl, JO-1Deforming arthropathy : anti-Jo-1 Fat-suppressed MRI Muscle biopsy : Longitudinal & Transverse section

Treatment of myositis Step 1 : high-dose prednisone Step 2 : azathioprine, mycophenolate, or methotrexate for steroid-sparing effect Step 3 : IVIg Step 4 : trial with rituximab, cyclosporine, cyclophosphamide, or tacrolimus Patients with ILD may benefit from aggressive treatment with cyclophosphamide or tacrolimus

[ 목차 ] Overlap syndrome (vs UCTD, MCTD) Polymyositis(PM) PM with Interstitial lung disease

Respiratory muscle weakness Interstitial lung disease 다발성 관절염의 빈도가 높다 ESR, CRP level 이 높다 anti JO1 Ab (t-RNA synthetase) 양성 조갑모세혈관검사상 microangiopathy 발생이 현저 Drug-induced pneumonitis (e.g. methotrexate) may cause dyspnea, nonproductive cough, and aspiration pneumonia

Major Categories of Alveolar and Interstitial Inflammatory Lung Disease

질병 초기, 혹은 질병에 선행하여 발생, JO-1 lymphocytic interstitial pneumonitis 가 비교적 흔하다 ILD 는 드물고 대개 pul.vascular disease 90% 에서 arthritis 가 선행 scl-70 관련

질병 초기, 혹은 질병에 선행하여 발생, JO-1 lymphocytic interstitial pneumonitis 가 흔하다 ILD 는 드물고 대개 pul.vascular disease 90% 에서 arthritis 가 선행 scl-70 Q. Why PM ILD? (not RA ILD?) 관절통 발생 4 개월만에 폐병변이 발견되었기 때문에 RA 관련 ILD 로 볼 수 없다. Early PM 에 동반된 ILD 로 보는 것이 합당하다.

Nonspecific interstitial pneumonia (NSIP) : pathologic description of a chronic interstitial pneumonia that lacks the histopathologic features typical of UIP, DIP, RB-ILD or AIP Inflammation dominantfibrosis dominant

more effective. IVCYC is effective Tx& more effective in early stage

Arthritis & Rheumatism 59(5) 2008, 677– PFTs improved with treatment in the early stages of disease 2. anti–Jo-1 Ab : predictive of ILD & positive prognostic factor