| Systemic
Sclerosis: Limited Scleroderma |
| This page was written
by Shelley Ensz,
and has not yet been medically edited. See Disclaimer. |
|
| What is Limited Scleroderma? |
| Systemic
Sclerosis (scleroderma) is classified in terms of the
degree and location of the skin involvement: Diffuse,
Limited, or CREST
Syndrome. |
| • CREST
Syndrome does not necessarily have any skin tightening
at all. |
| • Limited is limited to the
hands (although the face and neck may also be involved.) |
| • Diffuse is
when skin tightening also occurs above the wrists (or elbows,
see below). |
| • Systemic Sclerosis sans
Scleroderma is when there is internal organ fibrosis, but no
skin tightening. |
| Predictors of Intrinsic Pulmonary Arterial Hypertension (IPAH)
in Patients with Limited Cutaneous Systemic Sclerosis (lcSSc). LcSSc
patients with onset of Raynaud phenomenon at an older age,
low DLCO (carbon monoxide diffusing capacity) and/or history
of scleroderma renal crisis should be regarded as having
an increased risk of developing IPAH and should be monitored
closely for this complication.Tatiana Rodriguez-Reyna.
1861/533 ACR 2006. (Also see: Pulmonary
Hypertension) |
| Systemic
sclerosis-associated Sjogren's syndrome and relationship
to the limited cutaneous subtype: Results of a prospective
study of sicca syndrome in 133 consecutive patients. There
was a 68% prevalence of sicca syndrome in this prospective
series of SSc patients. PubMed. Arthritis Rheum. 2006
Jun 26;54(7):2243-2249. (Also see: Sjogrens) |
| Diagnosis of Limited
Scleroderma |
| Limited Scleroderma is diagnosed
when there is tight skin limited to the fingers, along with
either pitting
digital ulcers (secondary to Raynaud's) and/or lung
fibrosis. This is considered to be a "milder" form
of Scleroderma. The skin of the face and neck may also be involved
in Limited Scleroderma. |
| Limited Scleroderma often causes
Raynaud's and esophageal
problems. Occasionally other internal organ involvement
occurs, but usually only after many years of the disease. The
onset and progression of Limited Scleroderma is usually very
slow, and the outlook in general is very good.(1) |
| Using a self-reported functional score (FS) to assess disease progression in systemic sclerosis (SSc). The FS is a disease-specific, inexpensive and practical instrument for assessing functional status in SSc. It is a promising self-administered assessment tool for use in evaluating new SSc treatments. Rheumatology Volume 46, Number 7 Pp. 1107-1110. (Also see: Diffuse Scleroderma and CREST) |
| Association
of Polymorphisms in the IL1B and IL2 Genes with Susceptibility
and Severity of Systemic Sclerosis. IL1B and IL2 gene
polymorphisms may be involved in susceptibility to SSc. Moreover,
the IL2-384-G allele may be a marker for the limited phenotype
of SSc. J Rheumatol 2007 May;34:997-1004. (Also see: Causes
of Scleroderma: Genetics) |
| Increased
Serum Soluble CD40 Levels in Patients with Systemic Sclerosis. Elevated
serum sCD40 levels were associated with lcSSc. These results
suggest that the blockade of CD40/CD40 ligand interaction
could be a potential therapeutic strategy in SSc. J Rheumatol
2007 February;34:353–8. (Also see: Causes
of Scleroderma) |
| Urticarial
vasculitis appearing in the progression of systemic sclerosis
(SSc). We demonstrate that, in the present case, mast
cells might be involved in both courses of urticarial vasculitis
and SSc as a common factor. PubMed. J Dermatol. 2006 Nov;33(11):792-7.
(Also see: Vasculitis, Skin
Fibrosis, and Causes
of Scleroderma: Molecular Defect) |
| Prevalence
of Sicca Symptoms and Sjogren's Syndrome in Systemic Sclerosis:
Results of a Prospective Two-Center Series of 133 Patients. The
prevalence of sicca symptoms and SS, using international
classification criteria for SS, has been found respectively
in 68% and 14% of SSc patients. J. Avouac. FRI0318 EULAR
2006. (Also see: Sjogren's
Syndrome) |
| Clinical
and Immunological Features in Patients with Systemic Sclerosis. There
are differences in clinical and immunological findings between
diffuse scleroderma (dSSc) and limited scleroderma (lSSc):
severe capillary damage, arthralgia, muscle weakness, tendon
friction rubs, joint contractures, esophageal, pulmonary,
cardiac and renal involvement are more common in patients
with dSSc. S. Arsik. AB0194 EULAR 2005. (Also see: Types
of Scleroderma and Diffuse
Scleroderma) |
| Anticentromere
Antibodies in Connective Tissue Diseases. ACA are very
specific of scleroderma (95.3%), in particular of lcSSc (98.5%),
but the PPV (positive predictive value) is low (51.3%). Indeed,
only 23% of patients with lcSSc had positive ACA. Our data
show that ACA may be detected in other CTD at a lower percentage. Carlos
M. González. 1686/509. ACR 2004. (Also see: Antibodies) |
| Anticentromere
Antibodies in Systemic Connective Inflammatory Disease. ACA
are very specific of scleroderma, in particular localized
cutaneous systemic sclerosis, but the positive predictive
value is low. ACA are detected only in 23 % of the patients
with localized cutaneous systemic sclerosis and can appear
in other systemic connective inflammatory disease. M.
Montoro Álvarez. AB0116 EULAR 2004. (Also see:Antibodies) |
| Different
Clinical Features in Patients with Limited and Diffuse Systemic
Sclerosis. Loss of capillaries (detected by nailfold
capillaroscopy), digital ulcers, interstital lung fibrosis,
degreased FVC and DLCO, esophageal hypomotility, musculoskeletal
impairment, heart and renal involvement are more common in
patients with diffuse scleroderma. Fingertip osteolysis,
telangiectasia and arthritis are equally frequent in both
forms of disease. P. Ostojic. FRI0290 EULAR 2004. (Also
see: Diffuse
Scleroderma) |
| Scleroderma-clinical
and pathological advances. Skin sclerosis is no longer
regarded as mandatory for the diagnosis of systemic sclerosis.
It is likely that clinical trials performed over the next
few years will transform the management of systemic sclerosis
and help to dispel its reputation as one of the least treatable
of the autoimmune rheumatic diseases. PubMed. Best Pract
Res Clin Rheumatol. 2004 Jun;18(3):271-90. (Also see: Diffuse
Scleroderma and Prof.
Carol Black) |
| Circulating
endothelial cells (CECs) as a marker of ongoing vascular
disease in systemic sclerosis (SSc). The presence of
CECs in patients with SSc may represent direct evidence of
endothelial disease and may be a promising new clinical marker
for active SSc. Notably, the association between CECs and
pulmonary hypertension and impaired carbon monoxide diffusing
capacity was evident in patients with limited cutaneous SSc
only. PubMed. Arthritis Rheum. 2004 Apr;50(4):1296-304.
(Also see: Pulmonary
Hypertension) |
| Research for Limited
Scleroderma |
| Scleroderma
Treatments and Clinical Trials. ISN. |
 Scleroderma
Care and Research Journal (PDF) This inaugural issue
for physicians focuses on elevating the standards of care
for scleroderma lung involvement. Articles include Interstitial
Lung Disease in Systemic Sclerosis: Optimizing Evaluation
and Management, as well as Pulmonary Hypertension
Related to Systemic Sclerosis: A Primer for the Rheumatologist. Journal
of the Scleroderma Clinical Trials Consortium (SCTC) Vol
1, No. 1, Autumn 2003 (Also see: Pulmonary
Hypertension) |
| Pneumatosis
cystoides intestinalis (PCI) with systemic sclerosis, limited
type resulting in a poor prognosis. PCI is rarely complicated
with SSc, and all cases previously reported were associated
with diffuse SSc. PubMed. Am J Med Sci. 2006 Aug;332(2):100-2.
(Also see: Gastrointestinal
Involvement) |
| A
clue for telangiectasis in systemic sclerosis: elevated serum
soluble endoglin levels in patients with the limited cutaneous
form of the disease. Patients with elevated sEndoglin
levels had telangiectasia more frequently than those with
normal sEndoglin levels. PubMed. Dermatology. 2006;213(2):88-92.
(Also see: Telangiectasia, Pulmonary
Hypertension, and Causes
of Scleroderma: Molecular Defect) |
| Daily
occupations and well-being in women with limited cutaneous
systemic sclerosis. Loss of occupations, low satisfaction
with leisure, perceived fatigue, shortness of breath, and
pain indicated poorer well-being in women with scleroderma
and need to be focused on in occupational therapy interventions. PubMed.
Am J Occup Ther. 2005 Jul-Aug;59(4):390-7. (Also see: Emotional
Adjustment) |
| Association
of limited scleroderma and pulmonary hypertension in a patient
with primary biliary cirrhosis. Sclerodactyly and telangiectasia
of the fingers and chest wall were found. PubMed. J Nippon
Med Sch. 2005 Aug;72(4):230-5. (Also see: Liver
Involvementand Pulmonary
Hypertension) |
| Prevalence
of Elevated Pulmonary Arterial Pressures Measured by Echocardiography
in a Multicenter Study of Patients with Systemic Sclerosis. Elevated
pulmonary arterial pressures (PAP) are common in both limited
and diffuse SSc disease, occurring in 21% of limited and
26% of diffuse SSc patients. A high index of suspicion is
important and routine echocardiography in symptomatic patients
may allow earlier diagnosis of PAH and intervention. J
Rheumatol. 2005 July;32:1273-8. (Also see: Pulmonary
Hypertension, Diffuse
Scleroderma, and ISN
Medical Advisory Board: Dr. Janet Pope) |
| Clonal
T cells in the blood of patients with systemic sclerosis. Clonally
expanded T cells were more commonly detected in patients
with limited cutaneous sclerosis than in those with diffuse
cutaneous sclerosis, which is also in accordance with a possible
role of clonal T cells in patients with limited cutaneous
sclerosis. PubMed. Arch Dermatol. 2005 Jan;141(1):88-9.
(Also see: Causes
of Scleroderma: T Cells) |
| Peripheral
nervous system in limited systemic sclerosis. Peripheral
nervous system (PNS) lesions are different in severity in
limited systemic sclerosis (lSSc) according to the disease
duration, being more severe in advanced than in early phase. PubMed.
Reumatismo. 2004 Oct-Dec;56(4):247-52. |
| Calcinosis:
Treatment of cutaneous calcinosis in limited systemic sclerosis
with minocycline. Minocycline may be effective in the
control of calcinosis in systemic sclerosis. PubMed. Ann
Rheum Dis 2003 Mar;62(3):267-9 (Also see: Skin
Involvement: Calcinosis) |
| Glomerulonephritis:
Anti-neutrophil cytoplasmic autoantibody-associated rapid
progressive glomerulonephritis complicated with both limited
and diffuse scleroderma. PubMed. Nihon Rinsho Meneki
Gakkai Kaishi 2002 Dec;25(6):473-9 (Also see: Diffuse
Scleroderma) |
| Pupil
Diameter: Pupillocynetic Activity of Substance P in Systemic
Sclerosis. Our results show that PBD (Pupil basal diameter)
is reduced in patients with SSc and that SP (Substance P)
induces a more intense myosis in SSc than controls. Moreover,
in lSSc PBD is lower and SP increases the myosis in lSSc
compared to dSSc and controls. This suggests a peculiar dysfunction
of PNS (peripheral nervous system) in patients with the limited
subset of SSc. J Rheumatol NO. 6 JUNE 2003;30:1231-7. |
| Limited Scleroderma
Patient & Caregiver Stories |
| Amy
B: Limited Scleroderma with CREST I am a full time
pharmacist in a pediatric hospital. In February or March
of this year, I began to be very intolerable of the cold... |
| Brenda:
Limited Scleroderma, CREST, Raynaud's I was sick for
five or six years, telling doctors how much pain I was in... |
| Christene:
Limited Scleroderma I was diagnosed when I was just
nineteen and at that time it was one of the scariest moments
of my life... |
| Circadiano:
Limited Scleroderma with Raynaud's in One Finger (Italy)
About ten years ago she was diagnosed with lupus.... |
| D.M.
Hoffe: Progressive Systemic Sclerosis (Scleroderma CREST) After
an endoscopy it was discovered I had a Watermelon Stomach... |
| Elaine
GH: Limited Systemic Sclerosis I tell everyone I meet
about scleroderma. I have joined two support groups and bought
all three editions of Voices of Scleroderma... |
| Helen
P: CREST Scleroderma I was diagnosed ten years ago
when the symptoms kicked in with a vengeance following a
very traumatic personal tragedy... |
| Iceback:
CREST or Limited Scleroderma Never having heard of
scleroderma, I never really asked any questions when he told
me and went home to look it up on the Internet... |
| Irja:
CREST, Limited Scleroderma I'm a 34-year-old woman
from the land of Santa Claus, Finland. Ive had CREST since
1998... |
| Jackie:
Scleroderma, CREST, and Pulmonary Hypertension We
were identical twins, and she died in 1988 from kidney failure.
We both started with Raynaud's... |
| Jenny:
Severe CREST Syndrome and Limited Scleroderma I completed
my last marathon in January 2002. On the advice of my doctors,
I will not continue to run such long distances... |
| Jo
P: Limited Scleroderma and Raynaud's I could barely
walk ten feet without needing to sit down... |
| Kath:
Systemic Sclerosis (Scleroderma) Moving to Spain has
had a positive effect on my Raynaud's, but has had no effect,
so far as I am aware, on my scleroderma... |
| Kathy
M: CREST (Limited Systemic Scleroderma) If you do
not have the right doctor, do not get furious, move on... |
| Kerrie:
CREST Syndrome with Limited Scleroderma If I was not
stressed before I went in, I certainly was then. I am going
to get referred on to see someone different. Hopefully, I
will be luckier second time around... |
| Leeben:
Limited Scleroderma and CREST I won't go into how
many doctors I had to visit before someone took me seriously;
it was a total nightmare... |
| Lisa
C: Limited Scleroderma My mother has rheumatoid arthritis
(RA) and my thirteen-year-old daughter has what is believed
to be early lupus... |
| Lorrie:
Limited Scleroderma/CREST At first I thought, "Oh
my goodness! I am going to die." But my doctor assured
me that I could very likely live to be an old woman... |
| Maria:
Scleroderma and Raynaud's (Ukraine) I live in a small
town and in a poor country. My doctors have no computers
or medical magazines... |
| Melinda:
Granddaughter of Systemic Scleroderma Patient My grandmother
was diagnosed with systemic scleroderma approximately twenty-five
years ago... |
| Rosie:
Limited Systemic Sclerosis (Australia) Some of my
symptoms may not be due to limited scleroderma, however most
of these symptoms have appeared since my diagnosis... |
| Rouge:
Limited Systemic Sclerosis (Italy) I am 35 years old,
and up to two years ago I thought I was only suffering from
Raynaud's Phenomenon... |
| Sharon:
CREST with Limited Scleroderma and Sjögren's (Australia)
Day by day I discover another food I cannot eat, as I get
heartburn (Esophageal Dysfunction) and my mouth and my tongue
burn. I cannot eat any fruit or fruit juices... |
| Sheila:
CREST with Limited Scleroderma I felt really scared,
confused, and dehumanized in the process of my diagnosis
and further dealings with the "western medical field".. |
| Shirley
Wright: Limited Scleroderma I have had this disease
for over twenty years now and I plan on being around a lot
longer... |
| Soose:
Limited Scleroderma I may have had scleroderma for
the last year and a half or two years and did not know it... |
| Susan
Raby-Dunne: Surviving Daughter of Scleroderma Patient My
mother lived a fairly full and productive life for over thirty
years after her diagnosis. She danced with my father and
they were accomplished ballroom dancers... |
| Tara:
Systemic Scleroderma I was diagnosed with scleroderma
in January 2000, but I have had it since April 1997. It only
became worse in the new year... |
| Theresa
C: CREST/Limited Scleroderma I am fifty years old
and I was diagnosed with CREST in September of 1996... |
| Tui:
Limited Systemic Scleroderma I am wondering whether
such treatments will be ongoing? |
| References |
| (1) Learning About Scleroderma. North
East Valley Division of General Practice, Victoria. |
