Successful treatment using rituximab in a patient with refractory polymyositis complicated by scleroderma renal crisis

BMJ Case Rep. 2017 Sep 23;2017:bcr2017221205. doi: 10.1136/bcr-2017-221205.

Abstract

Corticosteroids are the first-line treatment for patients with inflammatory myopathies. Myositis can be a clinical feature of scleroderma (polymyositis-scleroderma overlap syndrome), and treatment of this syndrome is a challenge for clinicians because moderate to high doses of corticosteroids are considered a risk factor for development of acute kidney injury in affected patients. We report here the case of a 56-year-old woman with scleroderma who developed polymyositis and was successfully treated with rituximab. Initial treatment of the polymyositis with prednisolone 40 mg/day was rapidly tapered to 2.5 mg/day due to development of scleroderma renal crisis, for which four weekly infusions of rituximab (500 mg; off-label) were given. She responded well to rituximab in addition to prednisolone 2.5 mg/day. Rituximab may improve inflammatory myopathies, even in cases where high-dose corticosteroids should be avoided due to complications. Rituximab should be considered as a treatment option in cases of refractory polymyositis.

Keywords: biological agents; connective tissue disease.

Publication types

  • Case Reports

MeSH terms

  • Antirheumatic Agents / administration & dosage
  • Antirheumatic Agents / therapeutic use
  • Churg-Strauss Syndrome / complications
  • Churg-Strauss Syndrome / diagnosis*
  • Churg-Strauss Syndrome / drug therapy
  • Diagnosis, Differential
  • Female
  • Humans
  • Hypertension, Renal / complications
  • Hypertension, Renal / diagnosis*
  • Hypertension, Renal / drug therapy
  • Infusions, Intravenous
  • Middle Aged
  • Polymyositis / complications
  • Polymyositis / diagnosis*
  • Polymyositis / drug therapy
  • Recovery of Function
  • Rituximab / administration & dosage
  • Rituximab / therapeutic use
  • Scleroderma, Localized / complications
  • Scleroderma, Localized / diagnosis*
  • Scleroderma, Localized / drug therapy
  • Severity of Illness Index

Substances

  • Antirheumatic Agents
  • Rituximab