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1 대한내과학회지 : 제 73 권부록 2 호 2007 임상강좌 만성류마티스질환에서급성악화의진료 가톨릭대학교의과대학내과학교실 민준기 Life-threatening conditions in rheumatic disorders Life-threatening illnesses in rheumatologic diseases -respiratory system- Exacerbation (flare-up) of pre-existing manifestations Development of new, life-threatening manifestations Infections resulting from immunosuppression Adverse effects of drugs used to treat autoimmune disorders Acute serious illnesses that are unrelated to the rheumatic disease Vocal cords Rheumatoid arthritis Juvenile rheumatoid arthritis Angioedema Subglottic obstruction Wegener s granulomatosis Tracheobronchial collapse Relapsing polychondritis Interstitial lung disease Rheumatoid arthritis, Scleroderma Systemic necrotizing vasculitis Dermatomyositis Drugs Methotrexate, Cyclophosphamide Pneumonia Opportunistic infections Tuberculosis, Pneumocystis carinii Aspergillus, Nocardia Cytomegalovirus, Pulmonary alveolar hemorrhage, Dermatomyositis Goodpasture s syndrome Wegener s granulomatosis Pulmonary hypertension Scleroderma Pleural effusions, Rheumatoid arthritis The five rheumatic diseases most frequently associated with pleuropulmonary disease (1) rheumatoid arthritis (2) systemic lupus erythematosus (3) systemic sclerosis (4) polymyositis/dermatomyositis (5) Sjögren syndrome 환자 : 38/ 남주소 : 호흡곤란현병력 : 1년전전신성홍반성루푸스진단, 최근약물복용중단 2 주전뺨발진, 구강궤양, 관절통발생이학적검사 : 혈압은 90/00 mm/hg, 맥박은 110회 / 분, 호흡수 20회 / 분, 체온 38 검사실소견 : 혈색소 (g/dl) 백혈구 (/mm 3 ) 혈소판 (/mm 3 ) 2주전 9.8 3, ,000 내원당일 7.2 2,800 96,000 방사선검사 : 경과 : 내원 2 병일째객혈발생 - S 754 -

2 - 민준기 : 만성류마티스질환에서급성악화의진료 - X-ray exacerbation or infection? Complement CRP Procalcitonin Classic triad of pulmonary hemorrhage anemia pulmonary infiltrates hemoptysis Diagnosis diffusion capacity (DLCO) other causes of hypoxia and dyspnea should be excluded. bronchoscopy Treatment immunosuppressants, plasmapheresis Conditions associated with pulmonary hemorrhage systemic lupus erythematosus Wegener s granulomatosis microscopic polyangiitis small-vessel vasculitis anti-glomerular basement membrane disease infection pulmonary hemosiderosis idiopathic 환자 : 39/ 여주소 : 호흡곤란현병력 : 1 년전신성경화증진단받고 prednisolone 7.5mg/ 일, bucillamine 200mg/ 일, aceclofenac sodium 200mg/ 일로치료받아오던중수일전부터상기증상발생 Hb (g/dl) platelet (/mm 3 ) Cr (mg/dl) bilirubin 1 st HD , th HD , LDH (U/L) UA: protein ++, blood culture: no growth, anti-gbm Ab, ACL, LAC Ab, Coomb s test: neg, schistocytes: pos - S 755 -

- 대한내과학회지 : 제 73 권부록 2 호 2007 - Clinicopathological findings in the subsets of pulmonary-renal

pulmonary-renal syndrome systemic lupus erythematosus, mixed cryoglobulinemia, Henoch-Schonlein

sclerosis Seizures Hypertensive encephalopathy Delirium/psychosis Infections CNS vasculitis

Giant cell arteritis Antiphospholipid syndrome, PAN Isolated CNS vasculitis -nervous system-

: Behcet s disease Polyneuropathy Guillain-Barre syndrome Necrotizing vasculitis Myopathy

좌측상하지근력약화현병력 : 약 10 년전베체트병진단후간헐적으로치료받아오던중수일전상기증상발생 MRI T1W T1W/CE T2W CSF findings: WBC: 8 /mm 3,

3 - 대한내과학회지 : 제 73 권부록 2 호 Clinicopathological findings in the subsets of pulmonary-renal syndrome in scleroderma June 12 th Sep 10 th Naniwa et al Mod Rheumatol:2007;17:37 44 pulmonary-renal syndrome systemic lupus erythematosus, mixed cryoglobulinemia, Henoch-Schonlein purpura, Wegener s granulomatosis, Microscopic polyangiitis, Goodpasture s syndrome systemic sclerosis Seizures Hypertensive encephalopathy Delirium/psychosis Infections CNS vasculitis Drugs: Corticosteroids Aseptic meningitis Drugs: NSAIDs Cerebral infarction Takayasu s disease Giant cell arteritis Antiphospholipid syndrome, PAN Isolated CNS vasculitis -nervous system- Cerebral hemorrhage Scleroderma Myelopathy Atlantoaxial dislocation: RA Noncompressive myelopathy : Behcet s disease Polyneuropathy Guillain-Barre syndrome Necrotizing vasculitis Myopathy Polymyositis-dermatomyositis Drugs Corticosteroids Neuromuscular blockade 환자 : 47/ 여주소 : 말늘어짐, 좌측상하지근력약화현병력 : 약 10 년전베체트병진단후간헐적으로치료받아오던중수일전상기증상발생 MRI T1W T1W/CE T2W CSF findings: WBC: 8 /mm 3, RBC: 0 /mm 3 protein: 25 (reference range, mg/dl) Glucose:48 mg/dl (serum glucose, 90 mg/dl). oligoclonal IgG band: negative. - S 756 -

- 민준기 : 만성류마티스질환에서급성악화의진료 - F/U MRI Primary neurological

subclincal NBS cerebral dural venous sinus thrombosis CNS

system involvement Sites of involvement in patients with NBD

14 Cerebral peduncle 11 Tegmentum 4 Quadrigeminal plate 1

Deep white matter 2 Basal ganglia 9 Caudate nucleus 3 Putamen

Spinal cord 2 Treatment of NBD high dose steroid,

agent T1W T1W/CE T2W 환자 : 28/ 여주소 : 의식저하현병력 : 전신성홍반성루푸스 2

CH50 11 U/ml, FANA 1:3200, homogenous 항 ds-dna 항체 > 50 IU/ml,

4 g/dl, WBC: 5,500/mm 3 platelet 299,000/mm 3 F/U CSF

4 - 민준기 : 만성류마티스질환에서급성악화의진료 - F/U MRI Primary neurological involvement in BS T1W T1W/CE T2W migranous headache subclincal NBS cerebral dural venous sinus thrombosis CNS involvement neuro-psycho-behcet s syndrome peripheral nervous system involvement Sites of involvement in patients with NBD Brainstem 18 Pons 14 Ventral portion 14 Tegmentum 9 Midbrain 14 Cerebral peduncle 11 Tegmentum 4 Quadrigeminal plate 1 Cerebral white matter 9 Subcortex 4 Peri-ventricular area 3 Deep white matter 2 Basal ganglia 9 Caudate nucleus 3 Putamen 7 Globus pallidus 4 Internal capsule 7 Thalamus 6 Meninges 2 Spinal cord 2 Treatment of NBD high dose steroid, cyclophosphamide, cyclosporine, azathioprine, TNF-α blocking agent T1W T1W/CE T2W 환자 : 28/ 여주소 : 의식저하현병력 : 전신성홍반성루푸스 2 년전진단, 1 개월전부터뺨발진, 구강궤양, 관절염증상발생 C3 67 mg/dl, C4 12 mg/dl, CH50 11 U/ml, FANA 1:3200, homogenous 항 ds-dna 항체 > 50 IU/ml, Hb: 11.4 g/dl, WBC: 5,500/mm 3 platelet 299,000/mm 3 F/U CSF findings: WBC: 1/mm 3, RBC: 5/mm 3 protein: 29(reference range, mg/dl) glucose: 54mg/dL (serum glucose, 86 mg/dl). oligoclonal IgG band: negative. - S 757 -

- 대한내과학회지 : 제 73 권부록 2 호 2007 - neuropsychiatric manifestations of Central nervous system Peripheral nervous system Aseptic meningitis Acute inflammatory demyelinating Cerebrovascular disease

Movement disorder (chorea) Myasthenia gravis Myelopathy Cranial neuropathy Seizure disorder Plexopathy Acute confusional state Polyneuropathy Anxiety disorder Cognitive dysfunction Mood disorder

3 g/dl, WBC: 7,900/mm 3 platelet 69,000/mm 3 before pregnancy at admission ACL (IgG) : 26 PL 43 PL ACL (IgM): 7 PL 14 PL Lupus anticoagulant: positive positive β2-gpi Ab: ND 11.

thrombosis, occurring within any tissue or organ Complications of pregnancy One or more unexplained deaths of morphologically normal fetuses at or after the 10th week of gestation; or One or more

Laboratory Criteria Anticardiolipin antibodies, Lupus anticoagulant antibodies A diagnosis of definite APS requires the presence of at least one of the clinical criteria and at least one of the

5 - 대한내과학회지 : 제 73 권부록 2 호 neuropsychiatric manifestations of Central nervous system Peripheral nervous system Aseptic meningitis Acute inflammatory demyelinating Cerebrovascular disease polyradiculopathy Demyelinating syndrome (Guillain Barre syndrome) Headache (including migraine and Autonomic disorder idiopathic intracranial hypertension) Mononeuropathy, single or multiplex Movement disorder (chorea) Myasthenia gravis Myelopathy Cranial neuropathy Seizure disorder Plexopathy Acute confusional state Polyneuropathy Anxiety disorder Cognitive dysfunction Mood disorder Arthritis Rheum 1999;42: Psychosis 환자 : 24/ 여주소 : 좌측상하지근력약화현병력 : 약 7 년전전신성홍반성루푸스진단, 3 주전출산 C mg/dl, C mg/dl, CH50 11 U/ml, 항 ds-dna 항체 33.6 IU/ml, Hb: 12.3 g/dl, WBC: 7,900/mm 3 platelet 69,000/mm 3 before pregnancy at admission ACL (IgG) : 26 PL 43 PL ACL (IgM): 7 PL 14 PL Lupus anticoagulant: positive positive β2-gpi Ab: ND 11.0 TIW T2W TIW /CE DWI Criteria for the Classification of the Antiphospholipid Syndrome (APS) Clinical Criteria Vascular thrombosis One or more clinical episodes of arterial, venous, or small-vessel thrombosis, occurring within any tissue or organ Complications of pregnancy One or more unexplained deaths of morphologically normal fetuses at or after the 10th week of gestation; or One or more premature births of morphologically normal neonates at or before the 34th week of gestation; or Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation Laboratory Criteria Anticardiolipin antibodies, Lupus anticoagulant antibodies A diagnosis of definite APS requires the presence of at least one of the clinical criteria and at least one of the laboratory criteria. 환자 : 48/ 여주소 : 어지럽고넘어지려고함. 현병력 : 전신성홍반성루푸스 8년전진단받고 prednisolone 10mg/ 일복용중상기증상발생 Hb: 10.6 g/dl, WBC: 3,000/mm 3 (seg 86.5%, lym 8.1%, mono 5.4%) CD3: 71.1%, CD4:12.6%, CD8:70.3% C3 : mg/dl C4 : 2.82 mg/dl, CH50 < 5 U/mL FANA : 1:3200, anti-ds DNA Ab : >50 IU/mL CMV IgG/IgM (+/-), EBV IgG/IgM (+/-), VZV IgG/IgM (+/-), HIV (-) CSF findings: WBC: 0/mm 3, RBC: 0/mm 3, protein: 37(reference range, mg/dl), glucose: 52mg/dL (serum glucose, 76 mg/dl), oligoclonal IgG band: negative. - S 758 -

- 민준기 : 만성류마티스질환에서급성악화의진료 - T1W T1W/CE PCR for JC virus; CSF (-), biopsy specimen

particles Rheumatologic disease associated with progressive multifocal

virus characterized by progressive damage or inflammation of the white matter of

immune deficeincy 35 cases of PML in patients with rheumatic diseases Twenty-two

before the onset of neurologic symptoms was highly variable.

extensor plantar responses: absence decreased sensory below T4 level 검사실소견 : Hb: 7.

6 - 민준기 : 만성류마티스질환에서급성악화의진료 - T1W T1W/CE PCR for JC virus; CSF (-), biopsy specimen (+) brain biopsy T2W T2/FLAIR DW Luxol fast blue stain EM 33nm-sized viral particles Rheumatologic disease associated with progressive multifocal leukoencephalopathy (PML) cause of PML is a type of polyoma virus called the JC virus characterized by progressive damage or inflammation of the white matter of the brain at multiple locations It occurs almost exclusively in people with severe immune deficeincy 35 cases of PML in patients with rheumatic diseases Twenty-two of these were in patients The intensity of immunosuppression in the 6-month period before the onset of neurologic symptoms was highly variable. Leonard H et al Arthritis Rheum 2007:56; 환자 : 29/ 여주소 : 하반신마비현병력 : 전신성홍반성루푸스로 6개월전진단받고 prednisolone 7.5mg/ 일 hydroxychloroquine 300mg/ 일, 복용중상기증상발생 신경학적검사 : knee and ankle reflexes, extensor plantar responses: absence decreased sensory below T4 level 검사실소견 : Hb: 7.8 g/dl, WBC: 8,000/mm 3, platelet 253,000 /mm 3 C3/C4(mg/dl) 35/7, FANA : 1:3200, anti-ds DNA Ab : >50 IU/mL LAC, ACL Ab, β2-gpi Ab: neg CSF study: oigoclonal IgG band: negative. - S 759 -

- 대한내과학회지 : 제 73 권부록 2 호 2007 - transverse myelitis in T2W an acute or subacute inflammatory disorder of the spinal cord 1 2% of patients a discrete sensory level with lower limb weakness was the

7 - 대한내과학회지 : 제 73 권부록 2 호 transverse myelitis in T2W an acute or subacute inflammatory disorder of the spinal cord 1 2% of patients a discrete sensory level with lower limb weakness was the most common presenting feature. MRI is the diagnostic tool of choice strong association between TM in and the presence of apl corticosteroids and/or immunosuppression : methylprednisolone & cyclophosphamide anticoagulation remains controversial. acute transverse myelopathy in a patient with multiple sclerosis Coronary artery syndromes, Churg-Strauss granulomatosis Polyarteritis nodosa Kawasaki disease Wegener s granulomatosis Anticardiolipin antibody syndrome Myocarditis, PM/DM -cardiovascular system- Cardiac arrhythmias/conduction blocks Scleroderma, CREST syndrome Marantic endocarditis Valvular abnormalities RA, AS, Behcet s syndrome Reiter s syndrome Aortic aneurysm/dissection Rheumatoid arthritis Behcet s syndrome Takayasu s disease (aortoarteritis) Giant cell (temporal) arteritis Hypertensive emergencies Scleroderma, Juvenile rheumatoid arthritis with vasculitis Takayasu s disease (aortoarteritis) Pericardial effusion/tamponade Rheumatoid arthritis Mixed connective tissue disease 환자 : 26/ 여, 주소 : 호흡곤란현병력 : 2 개월전전신성홍반성루푸스진단받고 prednisolone 15mg/ 일복용중상기증상발생 Myocarditis clinical features 활력징후 : 혈압 100/70 mmhg, 맥박 125/ 분, 호흡수 20/ 분, 체온 38 청진소견 : gallop rhythm without murmur 검사실소견 : Creatine kinase 420 U/l (normal U/l). Chest radiography showed cardiomegaly with normal lung fields. EKG: T wave changes Echocardiogram: moderate mitral regurgitation, mild tricuspid regurgitation, global hypokinesia, left ventricular ejection fraction (LVEF): 36%. Fever, tachycardia out of proportion to fever, myalgias, headache, rigors Chest pain due to coexisting pericarditis Pericardial friction rub Severe cases may have CHF symptoms - S 760 -

- 민준기 : 만성류마티스질환에서급성악화의진료 - Myocarditis Diagnosis and Differential EKG-nonspecific changes, AV block, prolonged QRS suration, or ST elevation (with pericarditis) CXR-normal Cardiac Enzymes- may be

8 - 민준기 : 만성류마티스질환에서급성악화의진료 - Myocarditis Diagnosis and Differential EKG-nonspecific changes, AV block, prolonged QRS suration, or ST elevation (with pericarditis) CXR-normal Cardiac Enzymes- may be elevated Differentail-ischemia or infarct, valvular disease, and sepsis Myocarditis treatment immunosuppressants - steroid pulse therapy - azathioprine,cyclophosphamide - IVGV standard cardiac medications - salt restriction, digitalis, diuretic agents, - angiotensin-converting enzyme inhibitors Gastrointestinal bleeding Henoch-Schonlein purpura Polyarteritis nodosa Drugs: Anticoagulants, NSAIDs, Corticosteroids Secondary amyloidosis Pancreatitis Kawasaki disease Henoch-Schonlein purpura Drugs: Corticosteroids, Furosemide -gastrointestinal system- Hepatic failure Still s disease, Drugs Intestinal ischemic strictures/perforation Necrotizing vasculitis, Wegener s granulomatosis Essential mixed cryoglobulinemia Goodpasture s syndrome, Henoch-Schonlein purpura 환자 : 30/ 여주소 : 복통, 설사현병력 :2년전전신성홍반성루푸스진단, prednosolone 10mg 일 hydroxychloroquine 400mg/ 일복용중상기증상발생 Hb: 10.6 g/dl, WBC: 2,700/mm 3, platelet 120,000/mm 3 C3: 19.4 mg/dl C4: 2.5 mg/dl, CH50 < 5 U/mL FANA : 1:3200, anti-ds DNA Ab : >50 IU/mL ACL IgG/IgM: neg/neg, LAC: neg, β2-gpi: neg UA: protein (-), blood (-) CT scan of abdomen mesenteric vasculitis in 1 2% of patients with manifesting as abdominal pain rarely recurrent an acute gastrointestinal distress syndrome (AGDS): rapidly reverse IV methylprednisolone - S 761 -

- 대한내과학회지 : 제 73 권부록 2 호 2007 - -renal system- Renal crisis systemic sclwerosis Renovascular hypertension Aortoarteritis Scleroderma Interstitial nephritis Sjogren s syndrome -metabolic & endocrine

hemorrhage Autoimmune hemolytic anemia, Myelofibrosis Leukopenia Rheumatoid arthritis, Cytotoxic drugs Immune thrombocytopenia Pancytopenia Felty s syndrome Drug-induced marrow toxicity Cytotoxic

9 - 대한내과학회지 : 제 73 권부록 2 호 renal system- Renal crisis systemic sclwerosis Renovascular hypertension Aortoarteritis Scleroderma Interstitial nephritis Sjogren s syndrome -metabolic & endocrine system- Thyroiditis Hypothyroidism Sclerodrema Adrenal insufficiency Corticosteroid therapy Sudden omission Acute stress Hypoglycemia Drugs : hydroxychloroquine Severe anemia Gastrointestinal hemorrhage Autoimmune hemolytic anemia, Myelofibrosis Leukopenia Rheumatoid arthritis, Cytotoxic drugs Immune thrombocytopenia Pancytopenia Felty s syndrome Drug-induced marrow toxicity Cytotoxic drugs, Sulphasalazine -hematologic manifestations- Coagulopathy Autoantibodies against clotting factors: Lupus anticoagulant-hypoprothrombinemia syndrome : Thrombotic syndromes Antiphospholipid syndromes, Thrombotic thrombocytopenic purpura Reactive hemophagocytic syndrome Juvenile chronic arthritis, 환자 : 47/ 여주소 : 정신혼란현병력 : 전신성홍반성루푸스 1년전진단받고 prednisolone 10mg/ 일, hydroxychloroquine 400mg/ 일복용중상기증상발생활력징후 : 혈압 100/70 mmhg, 맥박 90/ 분, 호흡수 16/ 분체온 37.5 Hb: 7.1 g/dl, WBC: 4,300/mm 3, platelet 5,000 /mm 3, LDH: 2185 U/L, bilirubin: 2.7 mg/dl, haptoglobin: not detectable. C3 : 30.4 mg/dl C4 : 5.1 mg/dl, CH50 < 5 U/mL, FANA : 1:3200, anti-ds DNA Ab : >50 IU/mL serum creatinine,coagulation profile, fibrinogen degradation products, anticardiolipin antibodies, lupus anticoagulant: within normal limit Thrombotic Thrombocytopenic Purpura neurologic and renal abnormalities, fever thrombocytopenia. microangiopathic hemolytic anemia. reduced level of ADAMTS13. normal coagulation tests. elevated serum LDH. Brain MRI: a small ischemic cortical lesion on the left side. treatment of TTP emergently with large-volume plasmapheresis coupled with infusion of fresh-frozen plasma glucocorticoids Shah et al Nat Clin Pract Rheumatol. 2007;3: S 762 -

10 - 민준기 : 만성류마티스질환에서급성악화의진료 - treatment of acute flare Corticosteroids except: scleroderma, Kawasaki disease, Henoch-Schonlein purpura Cytotoxic drugs Plasmapheresis Intravenous immunoglobulin Angiotensin-converting enzyme inhibitors: scleroderma renal crisis - S 763 -

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ºÎÁ¤¸ÆV10N³»Áö case of brugada syndrome presented as chest pain STRCT rugada syndrome was described in 1992 as a new clinical entity characterized by electrocardiographic STsegment elevation in the right precordial leads