Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma

Rodnan, Gerald P.; Lipinski, Esther; Luksick, Joan

doi:10.1002/art.1780220205

Cited by 296 publications

(181 citation statements)

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“…This is due in part to the variability of the clinical manifestations, the lack of available objective measurements, and the paucity of controlled clinical trials. The major measure used for evaluation in the past has been skin thickness scoring, a semiquantitative technique introduced in the 1970s (9). However, only recently has a modified skin scoring method been developed and validated (2,3).…”

Section: Discussionmentioning

confidence: 99%

The value of the health assessment questionnaire and special patient‐generated scales to demonstrate change in systemic sclerosis patients over time

Steen

Medsger

1997

Arthritis & Rheumatism

424

328

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Objective. To determine the validity and usefulness of a modified Health Assessment Questionnaire (HAQ) for measurement of disease status and changes in disease status over time in patients with systemic sclerosis (SSc).Methods. Since 1985,1,250 patients attending the University of Pittsburgh Scleroderma Clinic have completed a modified HAQ annually. In addition to the standard HAQ questions about disability, the questionnaire includes visual analog scales (VAS) to evaluate SSc organ system symptoms, Raynaud's phenomenon, gastrointestinal (GI) tract and lung involvement, pain, and overall disease severity. In this study, the disability index (DI) (from the HAQ) and the VAS scores (on a 0-3 scale) were compared with various clinical and laboratory features recorded within 3 months of administration of the HAQ and VAS, using t-tests and Spearman's correlation tests.Results. The HAQ DI correlated directly with skin involvement, scleroderma heart or kidney disease, tendon friction rubs, hand contractures, and proximal muscle strength. Over time, the DI correlated with changes in skin score and was a good predictor of survival. There was a significant improvement in the DI during a 2-year time period in patients treated with D-penicillamine. The VAS for digital ulcers, GI symptoms, and lung symptoms correlated very closely with Submitted for publication September 9, 1996; accepted in revised form June 2, 1997. subjective and objective findings for these organ systems. The presence of new digital ulcers or improvement in digital ulcers showed significant associations with the Vascular scale, new GI symptoms or improvement in GI symptoms and institution of H,-blockers showed appropriate strong correlations with the GI scale, and changes in the forced vital capacity had an excellent correlation with the Lung scale (r = 0.58, P < 0.001). By Cox regression analysis, the HAQ DI was one of the best predictors of survival.Conclusion. These data provide convincing evidence that a self-administered questionnaire is an accurate and inexpensive tool to measure disease status changes in SSc. Prospective studies with the HAQ administered at regular intervals, as in controlled trials, should be performed to further assess the benefits and limitations of this instrument.

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Section: Discussionmentioning

confidence: 99%

The value of the health assessment questionnaire and special patient‐generated scales to demonstrate change in systemic sclerosis patients over time

Steen

Medsger

1997

Arthritis & Rheumatism

424

328

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“…Next to anthropometric and clinical data, bronchoalveolar lavage (BAL) fluid differentials and laboratory parameters were obtained. Skin score was measured by the modified Rodnan skin thickness score (MRSS) (19,20). Gas exchange at rest was assessed by the determination of hemoglobincorrected single-breath diffusing capacity for carbon monoxide (DL CO ) (21).…”

Section: Methodsmentioning

confidence: 99%

Soluble CD90 as a potential marker of pulmonary involvement in systemic sclerosis

Kollert¹,

Christoph²,

Probst³

et al. 2013

Arthritis Care & Research

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Objective. Vascular injury and endothelial cell (EC) activation are pathogenic hallmarks of systemic sclerosis (SSc; scleroderma). Human CD90 is highly expressed on activated ECs and can be shed from the cell surface. This study was conducted to examine whether soluble CD90 (sCD90) is elevated in the sera of patients with SSc and linked to pulmonary involvement and in particular, pulmonary arterial hypertension (PAH). Methods. sCD90 serum concentrations were assessed in 76 patients with SSc and related to clinical data, lung function, 6-minute walk distance, echocardiography, bronchoalveolar lavage fluid, and laboratory parameters. Thirty-one healthy volunteers and 29 patients with idiopathic retroperitoneal fibrosis (IRF) served as controls. Results. sCD90 serum concentrations were elevated in patients with SSc compared to healthy volunteers (P ‫؍‬ 0.001) and patients with IRF (P ‫؍‬ 0.01). SSc patients with pulmonary fibrosis (P ‫؍‬ 0.006) and patients with PAH (P < 0.001) had increased sCD90 serum concentrations compared to patients without the respective pulmonary manifestation of SSc. sCD90 levels correlated with diffusing capacity for carbon monoxide (n ‫؍‬ 65; r ‫؍‬ ؊0.348, P ‫؍‬ 0.005) and systolic pulmonary artery pressure (n ‫؍‬ 53; r ‫؍‬ 0.469, P < 0.001). Receiver operating characteristic curve testing determined an optimal cutoff value of >626 ng/ml with a sensitivity of 68% and a specificity of 83% for PAH (area under the curve 0.773, 95% confidence interval 0.648 -0.898; P < 0.001). Conclusion. sCD90 concentrations were increased in the sera of SSc patients, particularly in patients with vascular involvement of the lungs. These data suggest that sCD90 should be further evaluated as a marker for diagnosis of PAH in SSc.

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“…Objective histologic evaluation can be performed by measuring the amount of collagen in standard skin biopsy specimens. These methods consist of weighing and histometric methods (Dykes and Marks, 1977;Rodnan et al, 1979), which are inaccurate, and biochemical assays (Zachariae et al, 1989), which are elaborate.…”

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confidence: 99%

Dermal Organization in Scleroderma: The Fast Fourier Transform and the Laser Scatter Method Objectify Fibrosis in Nonlesional as well as Lesional Skin

Vries

Enomoto

Marle

et al. 2000

Laboratory Investigation

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SUMMARY:Scleroderma, a chronic, progressive disorder, is characterized by dermal fibrosis with collagen bundles orientated parallel to the epidermis. Simple objective parameters to evaluate disease progression and therapies are needed. We describe two methods, the laser scatter method and the fast Fourier transform (FFT), to measure collagen bundle orientation and spacing. Lesional sclerodermic skin (LS), nonlesional sclerodermic skin (nonLS), and control skin (CS) sections were evaluated for orientation ratio using the laser scatter method. The FFT was used to calculate orientation ratio, variation, and spacing of collagen bundles. Parameters were correlated with local and mean skin score measurements, on a scale of 0 (normal) to 3 (severely sclerotic). With both the laser scatter method and the FFT, orientation ratios of LS (respectively, 2.16 Ϯ 0.33 and 1.83 Ϯ 0.62) were significantly higher than CS (respectively, 1.70 Ϯ 0.35 and 1.38 Ϯ 0.15). NonLS orientation ratios (respectively, 1.92 Ϯ 0.15 and 1.48 Ϯ 0.44) were between LS and CS ratios. Orientation variation and bundle spacing of LS (respectively, 57.3 Ϯ 19.4 and 15.7 Ϯ 5.6 m) were significantly reduced compared to CS (respectively, 73.8 Ϯ 15.0 and 18.9 Ϯ 1.9 m). NonLS orientation ratios (respectively, 57.2 Ϯ 29.0 and 15.6 Ϯ 6.1 m) were similar to LS. Bundles in LS are more parallel, show less variation in orientation, and are more densely packed than in CS. There was a linear correlation between mean skin score and orientation ratio. Local skin score was not linearly correlated to orientation ratio. Our findings suggest that nonLS dermis without clinical sclerosis already shows fibrotic characteristics. Both techniques were easy to use and suitable for objectifying dermal fibrosis in scleroderma lesions. FFT is more accurate and reproducible than the laser scatter method and allows simultaneous pathological evaluation of the location of the analyzed tissue sections. Future studies will need to focus on the correlation between clinical disease severity and collagen bundle characteristics. (Lab Invest 2000, 80:1281-1289.

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Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma

Cited by 296 publications

References 16 publications

The value of the health assessment questionnaire and special patient‐generated scales to demonstrate change in systemic sclerosis patients over time

The value of the health assessment questionnaire and special patient‐generated scales to demonstrate change in systemic sclerosis patients over time

Soluble CD90 as a potential marker of pulmonary involvement in systemic sclerosis

Dermal Organization in Scleroderma: The Fast Fourier Transform and the Laser Scatter Method Objectify Fibrosis in Nonlesional as well as Lesional Skin

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