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Proper Care and Cleaning of the Microscope
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Microscopic polyangiitis with dermatomyositis.

Erin M Bauer1,2, Ernest Brahn1

  • 1Department of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, California, USA.

European Journal of Rheumatology
|January 9, 2018
PubMed
Summary
This summary is machine-generated.

This case study highlights a rare instance of dermatomyositis co-occurring with microscopic polyangiitis. Prompt evaluation of positive p-ANCA and renal insufficiency is crucial in dermatomyositis patients.

Keywords:
ANCA associated vasculitisDermatomyositismicroscopic polyangiitisrenal failure

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Area of Science:

  • Rheumatology
  • Nephrology
  • Autoimmune Diseases

Background:

  • Dermatomyositis is a rare autoimmune disorder characterized by muscle weakness and skin rashes.
  • Extramuscular manifestations are common, but renal involvement is atypical.
  • Microscopic polyangiitis is a rare autoimmune vasculitis that can affect the kidneys.

Purpose of the Study:

  • To report a unique case of concomitant dermatomyositis and microscopic polyangiitis.
  • To emphasize the importance of evaluating renal function and specific autoantibodies in dermatomyositis patients.

Main Methods:

  • Case report of a 62-year-old female with dermatomyositis symptoms.
  • Serologic evaluation including p-ANCA testing.
  • Renal biopsy to diagnose microscopic polyangiitis.
  • Treatment with cyclophosphamide and rituximab.

Main Results:

  • Patient presented with classic dermatomyositis symptoms but normal creatine kinase levels.
  • Positive p-ANCA was detected initially.
  • Renal biopsy confirmed microscopic polyangiitis, leading to renal insufficiency.
  • The patient achieved sustained remission for over 2 years with treatment.

Conclusions:

  • Dermatomyositis and microscopic polyangiitis can co-occur, despite their individual rarity.
  • Positive p-ANCA and developing renal insufficiency warrant urgent investigation in dermatomyositis patients.
  • This case underscores the need for comprehensive evaluation in complex autoimmune presentations.