| Systemic Sclerosis: Limited Scleroderma |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
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| 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) |
| 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) |
| 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) |
| Damage of cutaneous peripheral nervous system evolves differently according to the disease phase and subset of systemic sclerosis. In SSc, PNS ultrastructure damage is linked to the progression and severity of skin involvement. (PubMed) Rheumatology (Oxford). 2005 May;44(5):607-13. |
| Calcinosis in rheumatic diseases. While warfarin, colchicine, probenecid, bisphosphonates, diltiazem, minocycline, aluminum hydroxide, salicylate, surgical extirpation, and carbon dioxide laser therapies have been used, no treatment has convincingly prevented or reduced calcinosis. (PubMed) Semin Arthritis Rheum. 2005 Jun;34(6):805-12. (Also see: Calcinosis ) |
| 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. |
