General Medicine

Stem Cell Transplant May Hold Benefits for Systemic Sclerosis

Scleroderma is a rare autoimmune disease that leads to hardening of connective tissue; it is estimated that fewer than 500,000 people in the United States have scleroderma.17 The etiology of scleroderma remains unclear; however, many patients have a personal or family history of thyroid diseases and other autoimmune disorders. In one small study, 7 of 18 patients with systemic scleroderma had a familial history of thyroid disease and 8 of 18 had a thyreopathy.18 Because of the high prevalence of thyroid disorders in patients with systemic scleroderma, routine screening for thyroid disorders might be warranted, especially in patients with risk factors for thyroid disorders and/or atypical clinical follow-up.18 Despite being a heterogeneous disorder, almost all patients have skin involvement manifest as a loss of cutaneous elasticity followed by skin thickening.19 Hands and fingers are often the first areas affected. Calcinosis, pruritus from dry skin, and joint contracture are other common findings.19 Image credit: ISM / CID

Scleroderma is a rare autoimmune disease that leads to hardening of connective tissue; it is estimated that fewer than 500,000 people in the United States have scleroderma.17 The etiology of scleroderma remains unclear; however, many patients have a personal or family history of thyroid diseases and other autoimmune disorders. In one small study, 7 of 18 patients with systemic scleroderma had a familial history of thyroid disease and 8 of 18 had a thyreopathy.18 Because of the high prevalence of thyroid disorders in patients with systemic scleroderma, routine screening for thyroid disorders might be warranted, especially in patients with risk factors for thyroid disorders and/or atypical clinical follow-up.18

Despite being a heterogeneous disorder, almost all patients have skin involvement manifest as a loss of cutaneous elasticity followed by skin thickening.19 Hands and fingers are often the first areas affected. Calcinosis, pruritus from dry skin, and joint contracture are other common findings.19 Image credit: ISM / CID

In the absence of an effective pharmacotherapy for systemic sclerosis, researchers suggest stem cell transplant may have potential in the management of cutaneous and internal organ manifestations.

HealthDay News — Stem cell transplant may be an effective treatment for systemic sclerosis (SSc), according to a small study published online in the International Journal of Rheumatic Diseases.

Velu Nair, MD, from the Armed Forces Medical College in Pune, India, and colleagues assessed 4 patients who underwent autologous hematopoietic stem cell transplant (HSCT) for SSc between 2008 and 2012. Selection criteria included rapidly progressive disease with involvement of at least 1 major organ.

The researchers found that, over 7 years of follow-up, there was significant sustained improvement in skin score as well as in vasculopathy and gastrointestinal manifestations.

There was no further deterioration in interstitial lung disease. There was also remarkable improvement in quality of life indices among all subjects. No transplant-related complications were seen.

“In absence of an effective pharmacotherapy for SSc, autologous HSCT has a huge potential in management of cutaneous and internal organ manifestations,” the authors write.

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Reference

Nair V, Vasdev V, Kumar A, Shankar S, Nair V, Sharma A. Stem cell transplant in systemic sclerosis: an Indian experience [published online February 2, 2018]. Int J Rheum Dis. doi: 10.1111/1756-185X.13262

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