Skin thickness score as a predictor and correlate of outcome in systemic sclerosis: High-dose versus low-dose penicillamine trial

Clements, Philip J.; Hurwitz, Eric L.; Wong, Weng Kee; Seibold, James R.; Mayes, Maureen D.; White, Barbara; Wigley, Fredrick M.; Weisman, Michael H.; Barr, Walter G.; Moreland, Larry W.; Medsger, Thomas A.; Steen, Virginia D.; Martin, Richard W.; Collier, David H.; Weinstein, Arthur; Lally, Edward V.; Varga, John; Weiner, Saul J.; Andrews, Brian S.; Abeles, Moshe; Fürst, Daniel E.

doi:10.1002/1529-0131(200011)43:11<2445::aid-anr11>3.0.co;2-q

Cited by 283 publications

(210 citation statements)

References 13 publications

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“…Therefore, a large prospective cohort study is essential to confirm our findings; 2) our data did not include the modified Rodnan total skin thickness score, which is the most common technique for the measurement of skin involvement (Furst et al 1998;Clements et al 2000); 3) anti-RNA polymerase III antibody was examined only when patients had negative results for other autoantibodies; and 4) autoantibodies that can be detected in patients with SSc overlap syndrome (e.g., anti-PM-Scl antibody, anti-Ku antibody, and anti-RuvBL1/2) could not be examined at our institute (Kaji et al 2014). 5) Our conclusion would simply reflect that PM/DM-related ILD has a high risk of AE.…”

Section: Discussionmentioning

confidence: 77%

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

Tomiyama

Watanabe

Ishii

et al. 2016

Tohoku J. Exp. Med.

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Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by extensive fibrosis and autoantibodies. Its clinical manifestations are diverse and include Raynaud's phenomenon, gastrointestinal dysmotility, interstitial lung disease (ILD), pulmonary hypertension, and renal crisis. Among these, ILD is the primary cause of SSc-related death. It has been considered that acute exacerbation of ILD (AE-ILD) is not common in patients with SSc; however, little is known about the prevalence of AE-ILD in Japanese patients with SSc. In this study, we aimed to clarify the prevalence, clinical characteristics, and prognosis of patients with SSc who developed AE-ILD and to identify predictive factors for AE-ILD in our Japanese cohorts. Clinical data of patients who visited our department from 1990 to 2014 and fulfilled the 2013 classification criteria for SSc were retrospectively reviewed. A total of 139 patients were enrolled. The mean age of onset was 49.1 years, and 113 (81.3%) patients were female; 116 (83.5%) had limited cutaneous involvement, and the overall 10-year survival rate was 92.0%. Among 66 (47.5%) patients with ILD, 13 (9.4%) developed AE-ILD. Patients with AE-ILD had a significantly higher incidence of overlap with polymyositis (PM) or dermatomyositis (DM) and lower prevalence of anticentromere antibodies with higher mortality rate compared with those without AE-ILD. Multivariate Cox regression analysis identified that an overlap with PM or DM was the most significant predictive factor for AE-ILD. Our study results suggest that Japanese patients with SSc, particularly patients overlapped with PM or DM, have a high risk of AE-ILD.

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Section: Discussionmentioning

confidence: 77%

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

Tomiyama

Watanabe

Ishii

et al. 2016

Tohoku J. Exp. Med.

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“…There are 2 subtypes of SSc, i.e., diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc), according to the extent of cutaneous involvement (2); dcSSc has more extensive skin involvement, including the trunk or proximal extremities, and is associated with more internal organ involvement such as significant interstitial lung disease (ILD), cardiomyopathy, and scleroderma renal crisis (SRC) and with increased mortality (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”

Section: Introductionmentioning

confidence: 99%

Association of C‐reactive protein with high disease activity in systemic sclerosis: Results from the Canadian Scleroderma Research Group

Muangchan¹,

Harding²,

Khimdas³

et al. 2012

Arthritis Care & Research

101

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“…29% patients discontinued treatment due to toxicity. Recently it was seen that 125mg penicillamine on alternate days was as effective as high dose therapy, with no advantage achieved on increasing the dose beyond this [3][4][5]. The recommended duration of low dose therapy is 2 years.…”

Section: Discussionmentioning

confidence: 99%

“…However, laboratory monitoring at this dose is cumbersome and many side effects are seen [2]. Recently low dose penicillamine 125 mg on alternate days has been found to be as effective as high dose therapy with lesser side effects [3][4][5].…”

Section: Introductionmentioning

confidence: 99%

Low dose penicillamine in systemic sclerosis: is it effective?

Sameem¹,

Hassan²,

Majid³

et al. 2012

Our Dermatol Online

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Low dose D-penicillamine 150mg was given on alternate days to 23 patients of limited cutaneous systemic sclerosis (lcSSC) and 5 of diffuse cutaneous systemic sclerosis (dcSSC) subtypes. Modified Rodnan scoring remained unchanged in 19 and progressed in 3 patients of lcSSc. Only 1 female showed a decrease in the score. In the dcSSc, score decreased only in 1. She had a baseline score of 12 which went down to 4. No new systemic activity was seen in her. StreszczenieNiską dawkę D-penicylamine 150mg otrzymywało co drugi dzień 23 pacjentów z ograniczoną skórną postacią twardziny układowej (lcSSc) i 5 pacjentów z rozlaną skórną postacią twardziny układowej (dcSSC). Zmodyfikowana punktacja Rodnan pozostała niezmieniona w 19 przypadkach a postępowała u 3 chorych z lcSSc. Tylko jedna kobieta wykazała spadek w punktacji. W dcSSc, wynik zmniejszył się tylko w 1 przypadku. Pacjentka z 12 punktów bazowych ostatecznie otrzymała 4 punkty. Obserwowano u niej brak nowej ogólnoustrojowej aktywności choroby.

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Skin thickness score as a predictor and correlate of outcome in systemic sclerosis: High-dose versus low-dose penicillamine trial

Cited by 283 publications

References 13 publications

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

Association of C‐reactive protein with high disease activity in systemic sclerosis: Results from the Canadian Scleroderma Research Group

Low dose penicillamine in systemic sclerosis: is it effective?

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