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
Limited cutaneous
Diffuse cutaneous
Systemic sclerosis sine
scleroderma
Environmentally-induced
scleroderma
MCTD
Localized scleroderma
Linear scleroderma
En coup de sabre
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
superficial subcutaneous
calcification - linear
deeper subcutaneous -
amorphous, clump-like
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
thickened, sclerotic
fixed or tethered to
underlying
structures
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