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Systemic sclerosis

Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the generalised type of the disease, can be fatal. The localised type of the disease tends not to be fatal. The term 'localised, generalised sclerderma' can be used to describe cases where the disease covers a large area of the body - typically more than 40%. more...

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Signs and symptoms

Scleroderma affects the skin, and in more serious cases, it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. Typically the skin appears reddish or scaly in appearance. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage will weaken limbs and affect appearance.

The seriousness of the disease varies hugely between cases. The two most important factors to consider are, the level of internal involvement (beneath the skin), and the total area covered by the disease. For example there are cases where the patient has no more than one or two lesions (affected areas), perhaps covering a few inches. These are less serious cases and tend not to involve the internal bodily functions.

Cases with larger coverage are far more likely to affect the internal tissues and organs. Where an entire limb is affected, symptoms will almost certainly have serious consequences on the use of that limb. The heart and lungs will be affected when the disease covers this area of the torso. Some patients also experience gastrointestinal problems, including heartburn and acid reflux. Internal scarring may sometimes spread beyond what can be seen by the naked eye.

There is discoloration of the hands and feet in response to cold. Most patients (>80%) have Raynaud's phenomenon, a vascular symptom that can affect the fingers, and toes.

Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes, which are known as digital ulcers.

Types

There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.)

Diffuse scleroderma

Diffuse scleroderma is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening.

Limited scleroderma/CREST syndrome

The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.

The limited form is often referred to as "CREST" syndrome. CREST is an acronym for:

  • Calcinosis
  • Raynaud's syndrome
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia

These five are the major symptoms of the CREST syndrome.

Read more at Wikipedia.org


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The Loss of Endothelium-Dependent Vascular Tone Control in Systemic Sclerosis
From CHEST, 2/1/01 by Riccardo Livi

To the Editor:

Systemic sclerosis (SSc) is characterized by the widespread involvement of the microvasculature and by severe damage to the endothelium[1] as well as by the modification of vascular tone control.[2] The endothelium-dependent control of vascular tone is one of the main functions that is jeopardized during endothelial injury.

The work of Cailes et al[3] is a precious report showing the impairment of the endothelium-dependent control of the vascular tone in the lungs of SSc patients. Actually, our group was the first to report in 1990 such a result in the acral circulation of patients with SSc. We demonstrated that during the evolution of the disease, the endothelium-dependent vasodilation was impaired either when the level of von Willebrand factor, the marker of endothelial injury, was normal in the early phase of the disease or when it was greatly raised in the advanced phase of the disease.[4] The failure of endothelial-dependent regulation of vasodilation then was confirmed in the acral circulation by Bedarida et al[5] and in the skin circulation by La Civita et al.[6]

Recent data indirectly also demonstrate a defective vasodilating ability in the renal vasculature of SSc patients who have no sign of kidney involvement during standard clinical evaluations.[7] In fact, we have found that the renal functional reserve, or the ability of the kidney to increase its glomerular filtration rate (GFR) and effective plasma flow (ERPF) when challenged with a protein or an amino acid load is blunted in patients with SSc. During the amino acid load test, GFR, ERPF, and total renal vascular resistance (TRVR) remained substantially unchanged in 20 SSc patients ([mean [+ or -] SD] GFR, -2 [+ or -] 13.1 mL/min; ERPF, -4.1 [+ or -] 62.5 mL/min; and TRVR, 662 [+ or -] 2,215 dyne [multiplied by] [s.sup.-1] [cm.sup.-5]), whereas 10 healthy control subjects showed the expected significant changes in these parameters (GFR, 30.0 [+ or -] 13.3 mL/min [p [is less than] 0.001]; ERPF, 111.6 [+ or -] 39.6 mL/min [p [is less than] 0.001]; TRVR, -1,659 [+ or -] 559 dyne [multiplied by] [s.sup.-1] [multiplied by] [cm.sup.-5]5 [p [is less than] 0.004]). Only five of 20 SSc patients had a GFR increase comparable to that of the control subjects ([is greater than or equal to] 10%). The defective response was not dependent on the duration or the clinical subset of the disease, and it could be observed early in the course of the illness (median time interval from diagnosis, 18 months).

There is general agreement that the activation of the renal functional reserve depends on preglomerular vasodilation, and nitric oxide has been indicated as a major mediator of the phenomenon. Thus, the defective renal functional response to the protein load reflects an impaired endothelial-dependent vasodilation in the renal vasculature.[7]

In conclusion, the analysis of the literature of the last decade shows that in SSc, the endothelium-dependent control of the vascular tone is profoundly deranged in different organs. This evidence strongly suggests that the pivotal aim of the physician, in particular in the early phase of the disease, is the protection of the endothelium in order to keep its function at a steady level. This might allow an adequate tissular blood flow, thus avoiding the damage due to ischemia and reperfusion, as well as the further breakdown of vessel patency.

Correspondence to: Riccardo Livi, MD, PhD, Department of Internal Medicine, Section of Rheumatology and Nephrology, Viale Pieraccini, 18-50139 Firenze, Italy; e-mail: rlivi@unifi.it

REFERENCES

[1] Matucci Cerinic M, Kahaleh BM, LeRoy EC. The vascular involvement in systemic sclerosis. In: Furst D, Clements P, eds. Systemic sclerosis. Philadelphia, PA: Lea and Febiger, 1995; 153-174

[2] Matucci Cerinic M, Generini S, Pignone A. New approaches to Raynaud's phenomenon. Curr Opin Rheumatol 1997; 544:9-14

[3] Cailes J, Winter S, Du Bois RM, et al. Defective endothelially mediated pulmonary vasodilation in systemic sclerosis. Chest 1998; 114:178-184

[4] Matucci Cerinic M, Pietrini U, Marabini S. Local venomotor response to intravenous infusion of substance P and glyceryl trinitrate in systemic sclerosis. Clin Exp Rheumatol 1990; 8:561-564

[5] Bedarida G, Kim D, Blaschke TF, et al. Venodilation in Raynaud's disease. Lancet 1993; 342:1451-1454

[6] La Civita L, Rossi M, Vagheggini G, et al. Microvascular involvement in systemic sclerosis: laser Doppler evaluation of reactivity to acetylcholine and sodium nitroprusside by iontophoresis. Ann Rheum Dis 1998; 57:52-55

[7] Livi R, Teghini L, Pignone A, et al. Renal functional reserve is impaired in patients with systemic sclerosis without clinical signs of kidney involvement. 2000 Ann Rheum Dis (in press)

COPYRIGHT 2001 American College of Chest Physicians
COPYRIGHT 2001 Gale Group

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