Thymus-dependent (T) lymphocyte deficiency in progressive systemic sclerosis

Hughes, Pamela J.; Holt, Shirley; Rowell, N.R.; Dodd, Janice G.

doi:10.1111/j.1365-2133.1976.tb00855.x

Cited by 37 publications

(7 citation statements)

References 7 publications

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“…PPAR γ , which resides intracellularly and counters TGF-β fibrogenesis, is also an additional receptor for LPA ( 224 ). In addition to S1P being able to “disarm” Foxp3 Tregs mentioned above, S1P and LPA regulate the function, migration, and trafficking of all lymphoid cells and monocyte/macrophage/dendritic cells with S1P also being able to sequester T cells in the thymus and peripheral lymphoid organs, resulting in some instances in lymphopenia, which is frequently found in patients with SSc ( 225 – 227 ). By acting on APC, S1P and LPA each can suppress development of Th1 T-helper cells, but they have different effects on Th2 T-helper cells in that S1P suppresses their development while LPA fosters their development ( 228 ).…”

Section: Immune System In Ssc Pathogenesismentioning

confidence: 99%

Pathogenesis of Systemic Sclerosis

et al. 2015

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Systemic scleroderma (SSc) is one of the most complex systemic autoimmune diseases. It targets the vasculature, connective tissue-producing cells (namely fibroblasts/myofibroblasts), and components of the innate and adaptive immune systems. Clinical and pathologic manifestations of SSc are the result of: (1) innate/adaptive immune system abnormalities leading to production of autoantibodies and cell-mediated autoimmunity, (2) microvascular endothelial cell/small vessel fibroproliferative vasculopathy, and (3) fibroblast dysfunction generating excessive accumulation of collagen and other matrix components in skin and internal organs. All three of these processes interact and affect each other. The disease is heterogeneous in its clinical presentation that likely reflects different genetic or triggering factor (i.e., infection or environmental toxin) influences on the immune system, vasculature, and connective tissue cells. The roles played by other ubiquitous molecular entities (such as lysophospholipids, endocannabinoids, and their diverse receptors and vitamin D) in influencing the immune system, vasculature, and connective tissue cells are just beginning to be realized and studied and may provide insights into new therapeutic approaches to treat SSc.

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Section: Immune System In Ssc Pathogenesismentioning

confidence: 99%

Pathogenesis of Systemic Sclerosis

et al. 2015

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“…Disease severity scores for the CREST and systemic sclerosis patients were determined by the method of Hughes et a1 (25), which ascribes 1 point each to involvement of finger, proximal, or facial skin, and 3 points each to involvement of any of the major organ systems.…”

Section: Diagnostic Criteriamentioning

confidence: 99%

Clinical associations of anticentromere antibodies and antibodies to topoisomerase i. a study of 355 patients

Weiner

Earnshaw

Senécal

et al. 1988

Arthritis & Rheumatism

208

111

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Anticentromere antibodies (ACA) and anti-topoisomerase I (anti-top0 I) were assayed in serum samples from 355 patients: 89 with proximal scleroderma; 54 with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility , sclerodactyly , telangiectasias), without proximal scleroderma; 154 with primary and secondary Raynaud's disease; and 58 with other rheumatic diseases, without Raynaud's disease. Sera from healthy control subjects were also assayed. Using immunoblotting techniques, anti-top0 I was detected in 28% of the patients with proximal scleroderma; using immunodiffusion techniques, this antibody was found in only 20% of the same group of patients. Anti-top0 I and ACA were found primarily in patients with scleroderma, CREST syndrome, and Raynaud's phenomenon. ACA identified patients with less severe disease, whereas anti-top0 I identified patients with skin and cardiac involvement and patients with malignancies.Anticentromere antibodies (ACA) and antibodies to Scl-70 have been described in patients with

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“…An increase of the SLEDAI score ³4 was de®ned as disease exacerbation. Disease activity in patients with PSS was estimated using a disease severity scale [17]. Involvement of gut, lungs, heart, kidney, or CNS was scored 0 for``normal'' or 3 for``abnormal'', whereas sclerosis of the face, hands or trunk, sicca syndrome, arthralgia, or myalgia/myositis were each scored 1 (total score 0±21).…”

Section: Introductionmentioning

confidence: 99%

A longitudinal study of autoantibodies against central nervous system tissue and gangliosides in connective tissue diseases

Weiner¹,

Klein²,

Berg³

2000

Rheumatology International

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Our objective was to investigate longitudinally, antibodies against central nervous tissue (anti-CNS) derived from bovine brain and gangliosides GM 1, GD1a, GD1b, and GT1b in 91 patients with connective tissue diseases (systemic lupus erythematosus, n = 38; mixed connective tissue disease, n = 16; primary Sjogren's syndrome, n = 7; progressive systemic sclerosis, n= 13; polymyositis/dermatomyositis, n=4; overlap syndrome, n = 5; undifferentiated connective tissue disease, n = 8). Anti-CNS and anti-ganglioside antibodies, measured by enzyme-linked immunosorbent assay, were found in 73% and 63% of patients, respectively. Anti-CNS positive sera were also reactive in Western blotting in 74% of cases and recognized up to 14 different polypeptides from 29 to 130 kDa. Anti-CNS and anti-ganglioside antibodies reflected only in a limited extent the disease activity. In 27 of 58 patients, anti-CNS antibodies remained positive independently of disease activity and antibody levels did not correlate with the phases of exacerbations. A total of 36 of 60 anti-CNS-positive patients, in contrast to two of 22 anti-CNS-negative patients, had major neuropsychiatric manifestations (P < 0.001). Anti-ganglioside antibodies were not significantly associated with neuropsychiatric manifestations. In conclusion, our longitudinal data suggest that anti-CNS antibodies may be an important marker for the diagnosis of cerebral involvement in connective tissue diseases, but the pathogenic role of these autoantibodies remains to be determined.

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Thymus-dependent (T) lymphocyte deficiency in progressive systemic sclerosis

Cited by 37 publications

References 7 publications

Pathogenesis of Systemic Sclerosis

Pathogenesis of Systemic Sclerosis

Clinical associations of anticentromere antibodies and antibodies to topoisomerase i. a study of 355 patients

A longitudinal study of autoantibodies against central nervous system tissue and gangliosides in connective tissue diseases

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