The Symptom Burden Index: Development and Initial Findings from Use with Patients with Systemic Sclerosis

Author: Michael A. Kallen, Maureen D. Mayes, Yana L. Kriseman, Sofia B. de Achaval, Vanessa L. Cox and Maria E. Suarez-Almazor
Date Published: June-2010
Source:

Objective Our study had 3 aims: (1) to evaluate the functioning of the Symptom Burden Index (SBI) in patients with systemic sclerosis (SSc);

Antagonistic Effect of the Matricellular Signaling Protein CCN3 on TGF-β- and Wnt-Mediated Fibrillinogenesis in Systemic Sclerosis and Marfan Syndrome

Author:Raphael Lemaire, Giuseppina Farina, Julie Bayle, Michael Dimarzio, Sarah A Pendergrass, Ausra Milano, Michael L Whitfield and Robert Lafyatis
Date Published: February-2010
Source: 

Abnormal fibrillinogenesis is associated with connective tissue disorders (CTDs), including Marfan syndrome (MFS), systemic sclerosis (SSc) and Tight-skin (Tsk) mice. We have previously shown that TGF-β and Wnt stimulate fibrillin-1 assembly and that fibrillin-1 and the developmental regulator CCN3 are both highly increased in Tsk skin.

IFN regulatory factor 5 is required for disease development in the FcgammaRIIB-/-Yaa and FcgammaRIIB-/- mouse models of systemic lupus erythematosus.

Author: Richez C, Yasuda K, Bonegio RG, Watkins AA, Aprahamian T, Busto P, Richards RJ, Liu CL, Cheung R, Utz PJ, Marshak-Rothstein A, Rifkin IR.
Date Published: January-2010
Source: Pubmed

Polymorphisms in the transcription factor IFN regulatory factor 5 (IRF5) are strongly associated in human genetic studies with an increased risk of developing the autoimmune disease systemic lupus erythematosus.

Th1 transcription factor T-bet regulates the expression of Tim-3.

Author: Anderson AC, Lord GM, Dardalhon V, Lee DH, Sabatos CA, Glimcher LH, Kuchroo VK.
Date Published: January-2010
Source: Pubmed

Tim-3 (T cell immunoglobulin, mucin domain)-3 is a membrane protein expressed at late stages of IFN-gamma secreting CD4(+) T helper type 1 (Th1) cell differentiation and constitutively on dendritic cells (DC).

Rosiglitazone alleviates the persistent fibrotic phenotype of lesional skin scleroderma fibroblasts.

Author: Shi-Wen X, Eastwood M, Stratton RJ, Denton CP, Leask A, Abraham DJ.
Date Published: January-2010
Source: Pubmed

Objective. The transcription factor peroxisome proliferator-activated receptor (PPAR)-gamma plays an important role in controlling cell differentiation.

Lymphatic and blood vessels in scleroderma skin, a morphometric analysis.

Author: Rossi A, Sozio F, Sestini P, Renzoni EA, Khan K, Denton CP, Abraham DJ, Weber E.
Date Published: January-2010
Source: Pubmed

Vascular involvement is frequent in systemic sclerosis, but the role of the lymphatic vasculature is poorly known. Our aim was to evaluate lymphatic vessels in systemic sclerosis skin lesions.

Observational study of treatment outcome in early diffuse cutaneous systemic sclerosis.

Author: Herrick AL, Lunt M, Whidby N, Ennis H, Silman A, McHugh N, Denton CP.
Date Published: January-2010
Source: Pubmed

OBJECTIVE: Randomized clinical trials in early diffuse cutaneous systemic sclerosis (dcSSc) are challenging. We used an observational approach to estimate the relative effectiveness of different current treatment approaches, capturing entry and outcome data in a standardized way.

Echocardiography as an outcome measure in scleroderma-related pulmonary arterial hypertension: a systematic literature analysis by the EPOSS group.

Author: Kowal-Bielecka O, Avouac J, Pittrow D, Huscher D, Behrens F, Denton CP, Foeldvari I, Humbert M, Matucci-Cerinic M, Nash P, Opitz CF, Rubin LJ, Seibold JR, Strand V, Furst DE, Distler O; EPOSS Group.
Date Published: January-2010
Source: Pubmed

OBJECTIVE: To assess the validation status of echocardiography with continuous Doppler (echo-Doppler) as an outcome measure in pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc).

Medical Mystery Pieced Together

Author:
Date Published: January-2010
Source: The Boston Channel.com

A troubling medical puzzle in South Boston has finally been pieced together. A relatively small community has an alarming number of cases of a debilitating disease.

Darker-skinned patients with morphea and related diseases are as likely to benefit from ultraviolet (UV) light therapy as patients with lighter skin. Some clinicians had speculated that the increased level of melanin found in darker skin might impede UV-A1, but that does not appear to be the case.

Bob Roehr (Modern Medicine) Dermatology Times. 10/01/09.