Clinical improvement of nephrogenic systemic fibrosis after kidney transplantation

Abstract

Nephrogenic systemic fibrosis (NSF) has been observed with increased frequency in recent years. Progressive hardening of the skin advancing to severe woody induration and the development of thickened hyperpigmented plaques on the extremities and the trunk are the main clinical features. Further progression of the disease results in flexion contractures of the upper and lower extremities, resulting in immobilization and severe morbidity. In this study, we reviewed our experience with seven end-stage renal disease patients who were referred to our center between January 2004 and June 2005 for kidney transplant evaluation or for diagnosis and treatment of their deteriorating condition. Diagnosis in all patients was confirmed by skin and muscle biopsy. Three of these patients underwent renal transplantations, and softening of the skin and improved mobility of the joints was noted after kidney transplantation. Three of the four patients who remained on dialysis showed further deterioration of their NSF despite a trial of immunosuppressive therapy. Improvement after transplantation could be secondary to immunosuppression, increased renal clearance and/or more effective fluid management.

Cutaneous Manifestations of ESRD

“…Improving renal function seems to slow NSF progression and may improve the signs and symptoms of disease (96,105). Renal transplant for patients with NSF does not guarantee disease improvement, but the majority of cases has had successful outcomes, with skin softening and increased joint mobility (94,97,105,106). Treatments with limited benefit include oral corticosteroids, ultraviolet A phototherapy, plasmapheresis, sirolimus, high-dose intravenous Ig, methotrexate, and pentoxifylline (97,100,104,107).…”

Nephrogenic Systemic Fibrosis

“…Restoration of sufficient renal function is the primary goal when NSF is identified (51). This goal might be reached by recovery from AKI or by renal transplantation.…”

Nephrogenic systemic fibrosis: Clinical spectrum of disease

“…It seems to be important to transplant the patient as soon as possible to reverse the fibrosing process before the NSF becomes sufficiently established to lead to permanent and irreversible disability. An open question is whether immunosuppressive (IS) therapy plays a role in disease regression and whether there are differences among the different IS therapies (71).…”