Progressive Systemic Sclerosis Sine Scleroderma

Rodnan, Gerald P.; Fennell, Robert H.

doi:10.1001/jama.1962.03050210027006

Cited by 173 publications

(76 citation statements)

References 19 publications

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“…The subset, sometimes called "visceral scleroderma", was described for the first time in detail by Rodnan and Fennel in 1962, who also coined the term "progressive systemic sclerosis sine scleroderma". 5 The only earlier case was reported by Abrams et al 6 They published in 1954 a description of a patient with visceral scleroderma, but without detectable skin involvement. ssSSc is a rare form of the disease and its real prevalence is difficult to estimate.…”

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confidence: 99%

Systemic sclerosis sine scleroderma

Kucharz¹,

Kopeć-Mędrek²

2017

Adv Clin Exp Med

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Systemic sclerosis is a rare generalized disease with scleroderma, i.e. skin thickening as one of the most common symptoms. The disease has 2 main subsets, diffuse and limited forms. The subset known as systemic sclerosis sine scleroderma (ssSSc) is a very rare subset characterized by the total or partial absence of cutaneous manifestations of systemic sclerosis with the occurrence of internal organ involvement and serologic abnormalities. The classification of ssSSc into 3 groups was proposed. Type I (complete) is characterized by the lack of any cutaneous changes typical for the disease at least until systemic sclerosisrelated insufficiency of any internal organ occurs. Type II (incomplete) is characterized by the absence of sclerodactyly, but other cutaneous involvements (e.g. calcifications, telangiectasies, pitting scars) can be found. Type III (delayed) is characterized by clinical internal organ involvement typical for systemic sclerosis that has appeared before skin changes (complete or incomplete). In general, the demographic and clinical characteristics of the ssSSc patients suggest that they are similar to those with diffuse or limited form of the disease. Diagnosis of ssSSc still remains difficult and this disease form should be considered in all cases of unexplained fibrotic involvement of the internal organs.

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mentioning

confidence: 99%

Systemic sclerosis sine scleroderma

Kucharz¹,

Kopeć-Mędrek²

2017

Adv Clin Exp Med

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“…Our study indicates that the slow acetylator phenotype, which is found in more than 95% of patients with hydralazine-induced lupus (1,4,5), is not increased in frequency among patients with spontaneous lupus. Since spontaneous lupus is a heterogeneous disorder, the possibility remains that there may be a small proportion of patients in whom a slow acetylator phenotype might predispose to the induction of a lupus syndrome after exposure to environmental aromatic amines or hydrazines (6) Infrequency of anticentromere antibody in patients without systemic sclerosis and without Raynaud's phenomenon…”

Section: To the Editormentioning

confidence: 55%

“…The latter group consisted of 17 (of 35 patients) with SSc and limited skin scferosis, 5 (of 16 patients) with SSc without scleroderma (according to descriptions by Rodnan and Fennel [4] and Giordano et a1 [S]), and 2 (of 68 patients) with diffuse cutaneous SSc. One of these 2 patients died of sclerodermatous visceral involvement 4 yearq after disease onset.…”

Section: To the Editormentioning

confidence: 99%

Infrequency of anticentromere antibody in patients without systemic sclerosis and without Raynaud's phenomenon

Migliaresi

Picillo

Tirri

et al. 1987

Arthritis & Rheumatism

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Reply To the Editor:We appreciate Dr. Emery's comments. We are aware of 3 published studies of drug-induced lupus that include results of HLA typing (1-3). An increased frequency of the HLA-DR4 antigen was noted in only 1 of 2 studies of hydralazine-induced lupus. This association was not observed in the 1 study of procainamide-induced lupus. The frequency of the HLA-DR6Y antigen was increased among procainamide-treated patients who developed a lupus syndrome, but not among those who developed antinuclear antibodies without symptoms of lupus (3). (The absence of an HLA association in the appearance of antinuclear antibodies would be expected considering that almost all individuals who take procainamide develop such antibodies.) It appears that more studies are required before a specific genetic predisposition-defined by the HLA antigen system-for the development of drug-induced lupus can be confirmed.Drug-induced lupus and spontaneous systemic lupus erythematosus differ in their clinical and serologic features, although similarities in the 2 syndromes suggest that a common pathogenetic mechanism cannot be excluded. Our study indicates that the slow acetylator phenotype, which is found in more than 95% of patients with hydralazine-induced lupus (1,4,5), is not increased in frequency among patients with spontaneous lupus. Since spontaneous lupus is a heterogeneous disorder, the possibility remains that there may be a small proportion of patients in whom a slow acetylator phenotype might predispose to the induction of a lupus syndrome after exposure to environmental aromatic amines or hydrazines (6).

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“…These patients all had diffuse scleroderma with multisystem involvement including typical changes in the skin, joints, esophagus and lungs (17). The average dose of cyclophosphamide was 2 mg/kg/day, and the duration of treatment ranged from 2-25 months.…”

Section: Patients Studiedmentioning

confidence: 99%

Chromosome abnormalities from cyclophosphamide therapy in rheumatoid arthritis and progressive systemic sclerosis (scleroderma)

Tolchin¹,

Winkelstein²,

Rodnan³

et al. 1974

Arthritis & Rheumatism

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In patients with rheumatoid arthritis and scleroderma, the frequency of major chromosome abnormalities was appreciably increased by prolonged therapy with cyclophosphamide. Metaphases from 8 patients with rheumatoid arthritis, receiving this cytotoxic drug (0.5-2.0 mg/kg/day), showed a significantly greater frequency in hypodiploidy and chromosome type breaks than a comparable control series. In addition, 5 patients with scleroderma were studied before and after the administration of cyclophosphamide and a significant increase in the number of cytogenetic aberrations was observed during therapy.The alkylating agent cyclophosphamide appears to be an effective therapeutic agent in certain connective tissue disorders in-

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Progressive Systemic Sclerosis Sine Scleroderma

Cited by 173 publications

References 19 publications

Systemic sclerosis sine scleroderma

Systemic sclerosis sine scleroderma

Infrequency of anticentromere antibody in patients without systemic sclerosis and without Raynaud's phenomenon

Chromosome abnormalities from cyclophosphamide therapy in rheumatoid arthritis and progressive systemic sclerosis (scleroderma)

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