What is the prognosis of eosinophilic fasciitis (EF)?

Updated: Dec 24, 2020
  • Author: Peter M Henning, DO; Chief Editor: Herbert S Diamond, MD  more...
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Answer

A retrospective review found that clinical factors associated with persistent fibrosis included presence of morphealike skin lesions, younger age at onset, truncal involvement, and presence of dermal fibrosclerosis on histopathologic specimen. [5]

Loss of edema is usually the first clinical sign of improvement and can occur within 4 weeks of commencing treatment. Concurrently, the skin becomes softer, but 3-6 months may elapse before maximal reduction in induration and contractures is achieved. [14, 15]

While total resolution of the clinical signs can occur, some degree of induration remaining even after many months of corticosteroid therapy is not unusual.

A direct correlation does not always exist between clinical disease activity and laboratory findings. The eosinophil count and ESR usually return to reference ranges within 6-8 weeks, although the ESR prolongation and hypergammaglobulinemia may persist for up to 12 months. [14, 15]

Eventually, corticosteroid therapy can be withdrawn in many patients, without relapse occurring.

The development of aplastic anemia is a rare but grave complication. [34]  One study reported on 4 patients with eosinophilic fasciitis and severe aplastic anemia. In 3 cases, the aplastic anemia was refractory to conventional immunosuppressive therapy with antithymocyte globulin and cyclosporine. However, in 1 patient, rituximab displayed significant efficacy for both the skin and hematologic symptoms. In an additional 19 cases of eosinophilic fasciitis and aplastic anemia, corticosteroid regimens improved skin symptoms in 5 of 12 cases but were ineffective in the treatment of aplastic anemia in all but 1 case. Aplastic anemia was profound in 13 cases and was the cause of death in 8 cases. Only 5 patients achieved long-term remission. [35]


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