Nephrogenic systemic fibrosis: A brief review

Abstract

Nephrogenic systemic fibrosis is a fibrosing disorder of the skin that develops in patients with advanced renal failure. It mostly presents with progressive hardening or induration of the skin of the extremities. Systemic involvement is also known to occur in this entity. Exposure to gadolinium contrast for radiological evaluation has been identified as the offending agent. The condition is progressive and can be seriously disabling. Therapeutic options are limited and not rewarding in majority of the cases. Awareness of this entity is important so that proper precautionary measures can be taken at the earliest to ameliorate the condition.

The Clinical Manifestations and Efficacy of Different Treatments Used for Nephrogenic Systemic Fibrosis: A Systematic Review

“…Nephrogenic systemic fibrosis (NSF) is a rare scleroderma-like fibrosing disorder which occurs in majorly association with chronic kidney disease (CKD), most likely in patients of stages 4 and 5 chronic kidney disease (CKD), but sometimes also in patients with stage 3 CKD. 1–3 Majorly a cutaneous manifestation but also effecting mobility, NSF was first reported in 1997; in that study, all 15 patients were dialysis dependent, with varying levels of renal impairment. 1 , 4 Histological studies show characteristic CD 34 and procollagen-positive dermal spindle cells, as well as randomly arranged collagen fibers along with mucin deposition.…”

“… 1–3 Majorly a cutaneous manifestation but also effecting mobility, NSF was first reported in 1997; in that study, all 15 patients were dialysis dependent, with varying levels of renal impairment. 1 , 4 Histological studies show characteristic CD 34 and procollagen-positive dermal spindle cells, as well as randomly arranged collagen fibers along with mucin deposition. 1 , 5 Typically, patients present with pruritus and burning sensation of the skin, as well as swelling of the arms and legs.…”

“… 1 , 4 Histological studies show characteristic CD 34 and procollagen-positive dermal spindle cells, as well as randomly arranged collagen fibers along with mucin deposition. 1 , 5 Typically, patients present with pruritus and burning sensation of the skin, as well as swelling of the arms and legs. The induration of skin and development of contractures is accompanied by debilitating disability due to loss of range of motion.…”

“…The induration of skin and development of contractures is accompanied by debilitating disability due to loss of range of motion. 1 , 6 , 7 The disease shows no inclination towards a certain age group or gender but does show some level of occurrence in patients with renal disease exposed to gadolinium-based contrast agents (GBCA). 1 , 8 …”

Nephrogenic systemic fibrosis: A frivolous entity

“…The timing of the appearance of skin lesions following gadolinium-based contrast agents (GBCAs) exposure is controversial. It may vary from a few months to several years (1 d to 10 years)[ 3 ]. The course of skin lesions is usually unremitting and chronic.…”