系统性硬皮病影像学表现

Systemic Scleroderma Jenny Sun Gillian Lieberman, M.D. March 2002 Jennyy Sun Gillian Liberman, MD 2 The patient - Ms. H „ 66 y.o. female „ Scleroderma for 15 years „ Complications „ GERD „ Interstitial lung disease „ Joint involvement – hips and hands „ Calcinosis Jennyy Sun Gillian Liberman, MD 3 General information I Systemic scleroderma ƒ Limited cutaneous ƒ Diffuse cutaneous ƒ Systemic sclerosis sine scleroderma ƒ Environmentally-induced scleroderma ƒ MCTD Localized scleroderma ƒ Linear scleroderma ƒ En coup de sabre ƒ Morphea A heterogeneous group of conditions linked by thickened, sclerotic skin lesions: Jennyy Sun Gillian Liberman, MD 4 General Information II „ Incidence - 4 to 253 /million in the US „ Female:male – 3:1; child bearing age; African Americans „ Genetic factors – HLA-DQ7, -DR2, „ Serologic testing - anticentromere, antitopoisomerase-1, or anti-RNA polymerase Jennyy Sun Gillian Liberman, MD 5 General information III „ Risk factors: „ Infection - CMV, B. burgdorferi „ Environmental exposure - petroleum- based solvents, vinyl chloride, L- tryptophan „ Drugs - bleomycin, pentazocine, cocaine Jennyy Sun Gillian Liberman, MD 6 General information IV „ Affected organ systems: „ Skin „ Lungs „ GI „ Musculoskeletal system „ Cardiovascular system Jennyy Sun Gillian Liberman, MD 7 Cutaneous Manifestations „ Pruritus „ Edema „ Telangiectasia „ Calcinosis „ Sclerodactyly Jennyy Sun Gillian Liberman, MD 8 Calcinosis: Scleroderma Scleroderma - remains subcutaneous Ms. H – frontal and lateral plain films of the left knee: extensive cutaneous calcification Diffused subcutaneous calcification Jennyy Sun Gillian Liberman, MD 9 Contrast this with dermatomyocitis which is in the Ddx for calcinosis 10 Dermatomyocitis ƒ superficial subcutaneous calcification - linear ƒ deeper subcutaneous - amorphous, clump-like ƒ muscular calcification at later stage Adopted from ACR archive - frontal plain film of the abdomen of a patient with dermatomyocitis showing generalized calcinosis Jennyy Sun Gillian Liberman, MD 11 Sclerodactyly http://www.raynauds.demon.co.uk/ Clinical presentation ƒ thickened, sclerotic ƒ fixed or tethered to underlying structures ƒ contraction Jennyy Sun Gillian Liberman, MD 12 Sclerodactyly Courtesy of Ferris Hall, M.D., BIDMC - another case of sclerodactyly affecting the hands Radiological findings: „ loss of soft tissue „ contracture Jennyy Sun Gillian Liberman, MD 13 Sclerodactyly Courtesy of Ferris Hall, M.D., BIDMC - another patient with sclerodactyly causing contraction of the digits. Radiological findings: „ loss of soft tissue „ contracture Jennyy Sun Gillian Liberman, MD 14 Gastrointestinal system „ Esophagus dysmotility „ Gastric atony „ Small intestine dilatation „ Pseudo-diverticuli of the colon Jennyy Sun Gillian Liberman, MD 15 GI – esophageal dysmotility http://www.med.univ-rennes1.fr/cerf/iconocerf/D/MEDI-000899-D_0960-I3.GIF „ Scleroderma „ Widened LES „ Dilatation of the distal 2/3 of esophagus Jennyy Sun Gillian Liberman, MD 16 Contrast this with Achalasia which is in the Ddx for dilated esophagus. 17 Achalasia „ bird beak LES „ dilatation of esophagus Adopted from ACR archive – barium swallow showing failure of relaxation in a patient with achalasia Jennyy Sun Gillian Liberman, MD 18 GI - small intestine dilatation „ Scleroderma „ duodenum and jejunum „ Hide bound sign - lumenal dilatation of the small bowel with normal number of valvulae conniventes Adopted from ACR archive – a patient with scleroderma Jennyy Sun Gillian Liberman, MD 19 GI – small intestine dilatation „ Scleroderma „ Generalized dilatation „ Pseudo- obstruction Courtesy of Ferris Hall, M.D., BIDMC. – a patient with scleroderma presenting with marked dilatation of the small intestine Jennyy Sun Gillian Liberman, MD 20 Contrast this with Adynamic ileus and mechanical obstruction which are in the Ddx for dilated bowel. 21 Adynamic ileus „ dilatation „ air-fluid levels Adopted from ACR archive – a patient with postoperative ileus Jennyy Sun Gillian Liberman, MD 22 Mechanical Obstruction „ transition point Adopted from ACR archive – a patient with CA Jennyy Sun Gillian Liberman, MD 23 GI - colon „ Scleroderma „ wide- mouthed pseudo- diverticula Courtesy of Ferris Hall, M.D. BIDMC. – a patient with scleroderma presenting with wide-mouthed pseudodiverticula. The patient also has true diverticulosis. Jennyy Sun Gillian Liberman, MD 24 GI – colon „ Scleroderma „ dilatation „ Ahaustral colon Courtesy of Ferris Hall, M.D., BIDMC – a patient with scleroderma presenting dilatation of the colon on barium enema Jennyy Sun Gillian Liberman, MD 25 Pulmonary fibrosis „ Scleroderma „ Bi-basalar pulmonary fibrosis Courtesy of Ferris Hall, M.D., BIDMC – a patient with scleroderma presenting bibasilar pulmonary fibrosis.. Jennyy Sun Gillian Liberman, MD 26 Pulmonary fibrosis „ Scleroderma „ Bi-basalar pulmonary fibrosis „ ground-glass density Courtesy of Pillip Boisell, M.D., BIDMC – CT scan of a patient with scleroderma presenting bibasilar pulmonary fibrosis. Jennyy Sun Gillian Liberman, MD 27 Pulmonary hypertension „ Scleroderma „ enlargement of pulmonary vessels „ Doppler - tricuspid regurgitation Æ pulmonary artery pressures This Image, provided by Carol Black, M.D., is adopted from uptodate 2002 - a plain film of the chest showing pulmonary hypertension in a patient with scleroderma. Jennyy Sun Gillian Liberman, MD 28 Musculoskeletal system „ Scleroderma „ Periarticular inflammation and fibrosis „ Distal interphalangeal and first carpal- metacarpal joints Jennyy Sun Gillian Liberman, MD 29 Musculoskeletal system „ Scleroderma „ Periarticular inflammation and fibrosis „ Distal interphalangeal and first carpal- metacarpal joints „ Narrowing of the radiometacarpal joint Our patient – plain film of the wrist series showing joint narrowing and subcutanesou calcification Jennyy Sun Gillian Liberman, MD 30 Musculoskeletal – ddx for periarticular fibrosis „ Osteoarthritis „ Rheumatoid arthritis „ Ankylosing spondylitis „ Psoriasis „ Reiter’s „ IBD Jennyy Sun Gillian Liberman, MD 31 Cardiovascular system „ Cardiac – pericarditis, pericardial effusion, myocardial fibrosis, congestive heart failure, myocarditis, and arrhythmias „ Vascular „ Digital Raynaud’s „ Esophageal Raynaud’s „ Renal artery stenosis and scleroderma renal crisis Jennyy Sun Gillian Liberman, MD 32 Raynaud’s http://sclerodermalungstudy.medsch.ucla.edu „ White – arterial spasm „ Blue – acrocyanosis „ Red – reperfusion hyperemia Jennyy Sun Gillian Liberman, MD 33 Summary „ Uncommon autoimmune diseases of unknown etiology „ Multi-organ system involvement „ Female predilection „ Diagnosed base on sclerotic skin lesions and esophageal dilatation Jennyy Sun Gillian Liberman, MD 34 Reference „ American College of Radiology Archive „ Denton, CP, Black, CM, Korn, JH, de Crombrugghe, B.Systemic sclerosis: Current pathogenetic concepts and future prospects for targeted therapy. Lancet 1996, 347: 1453. „ Fundamentals of Diagnostic Radiology, William E. Brant and Clyde A. Helms, 1994 „ Gamut „ LeRoy, EC, Black, CM, Fleischmajer, R, et al. Scleroderma : classification, subsets and pathogenesis. J Rheumatol 1988, 15:202 „ Uptodate 2002 „ Wells , Au, Hansell, DM, Rubens, MB, et al. The predictive value of appearances on thin section computed tomography in fibrosing alveolitis. Am Rev Respir Dis 1993; 148:1076 Jennyy Sun Gillian Liberman, MD 35 Acknowledgement „ Larry Barbaras and Cara Lyn D’amour, our Webmasters „ Pamela Lepkowski „ Michael Larson „ Gillian Leiberman, M.D. „ Ferris Hall, M.D. „ Phil Boiselle, M.D. „ Zhen (Richard) Lu, M.D. „ Chad Brecher, M.D. Jennyy Sun Gillian Liberman, MD Systemic Scleroderma The patient - Ms. H General information I General Information II General information III General information IV Cutaneous Manifestations Calcinosis: Scleroderma Contrast this with dermatomyocitis which is �in the Ddx for calcinosis Dermatomyocitis Sclerodactyly Sclerodactyly Sclerodactyly Gastrointestinal system GI – esophageal dysmotility Contrast this with Achalasia which is in the Ddx �for dilated esophagus. Achalasia GI - small intestine dilatation GI – small intestine dilatation Contrast this with Adynamic �ileus and mechanical �obstruction which are �in the Ddx for dilated bowel. Adynamic ileus Mechanical Obstruction GI - colon GI – colon Pulmonary fibrosis Pulmonary fibrosis Pulmonary hypertension Musculoskeletal system Musculoskeletal system Musculoskeletal – ddx for periarticular fibrosis Cardiovascular system Raynaud’s Summary Reference Acknowledgement