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From the American College of Rheumatology

Rheumatology for Primary Care
  • Symptoms
    • Fever
    • Joint Pain
    • Muscle Pain and/or Weakness
    • Rashes
    • Raynaud’s Phenomenon
  • Diseases
    • ANCA-Associated Vasculitis
    • Behcet’s Syndrome
    • Crystalline Arthropathies
    • Drug and Medication-Induced Rheumatic Diseases
    • Giant Cell Arteritis
    • Henoch-Schönlein Purpura
    • IgG4-Related Disease
    • Juvenile Dermatomyositis
    • Juvenile Idiopathic Arthritis
    • Kawasaki Disease
    • Mixed Connective Tissue Disease
    • Multisystem Inflammatory Syndrome in Children
    • Myositis
    • Polyarteritis Nodosa
    • Polymyalgia Rheumatica
    • Rheumatoid Arthritis
    • Sarcoidosis
    • Sjogren’s Disease
    • Spondyloarthritis
    • Systemic Lupus Erythematosus
    • Systemic Sclerosis
  • Case Studies
    • ANCA-Associated Vasculitis
    • Behcet’s Syndrome
    • Crystalline Arthropathies
    • Giant Cell Arteritis
    • Henoch-Schönlein Purpura
    • IgG4-Related Disease
    • Juvenile Dermatomyositis
    • Juvenile Inflammatory Arthritis
    • Kawasaki Disease
    • Mixed Connective Tissue Disease
    • Myositis
    • Pediatric Fevers
    • Polyarteritis Nodosa
    • Polymalgia Rheumatica
    • Raynaud’s Phenomenon
    • Rheumatoid Arthritis
    • Sarcoidosis
    • Sicca/Sjogren’s Disease
    • Spondyloarthritis
    • Systemic Lupus Erythematosus
    • Systemic Sclerosis
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Juvenile Dermatomyositis Case Studies

Home ยป Juvenile Dermatomyositis Case Studies
Juvenile Dermatomyositis Case Studies
  • Case 1

  • Case 2

  • Case 1

A 5 yo F presents with several weeks of cough and shortness of breath in the context of several months of decreased appetite and weight loss.

Questions to Ask

  • Why is she weak? Is it due to malnutrition or is it muscular in nature?
  • How could this be connected to her shortness of breath?

Physical Exam

  • Lungs clear to auscultation
  • Mild erythema of bilateral cheeks
  • Muscle weakness of proximal upper and lower extremities

Diagnostic Workup

  • Labs: CK=193, AST=105, ALT=142, ESR=25
  • Chest x-ray: shows mild increase in interstitial lung disease (ILD) markers
  • O2 is 93% in clinic
  • BMI is 17

Referrals

  • Refer to pediatric rheumatology ASAP
  • Refer to physical therapy

Further Workup

In advance of patient getting appointment with rheumatology, order MRI of lower extremities and antibody testing.

  • MRI of lower extremities reveals diffuse myositis of thigh and calf muscles.
  • Antibody testing reveals +MDA5 antibody, which is associated with progressive interstitial lung disease.

Treatment & Management

  • Start on high dose steroids, methotrexate, and IVIG for ILD
  • Her weight, muscle strength, and shortness of breath all improve on this regimen.
  • Work on optimizing patient nutrition
  • Case 2

A 7 yo F with a history of eczema presents with 6 months of intermittent diarrhea, weight loss, and generalized weakness.

Questions to Ask

  • Does her rash seem consistent with eczema?
  • How does her growth curve look?
  • Is she weak in certain muscle groups?

Physical Exam

  • Raised rash over her knuckles and knees (see images)
  • She is unable to climb up onto the exam table without significant help, revealing proximal muscle weakness.

Diagnostic Workup

  • Labs: CK=447, AST=154, ALT=107, ESR=35, Albumin=3.0, CBC shows normocytic anemia

Referrals

  • Refer to pediatric rheumatology
  • Refer to physical therapy and occupational therapy

Further Workup

In advance of patient getting appointment with rheumatology, order MRI of lower extremities, spine x-ray, bone density scan, and antibody testing.

  • MRI of lower extremities reveals diffuse myositis of thigh and calf muscles.
  • Antibody testing reveals +TIF-1 gamma antibody.
  • Spine x-ray reveals vertebral fracture.
  • Bone density scan shows severe osteopenia.

Treatment & Management

  • Start on steroids, methotrexate, and hydroxychloroquine.
  • Start on bisphosphonate for osteopenia.
  • With both physical and occupational therapy, she is able to return to sports.
  • Her rash significantly improves of the next 2-3 months.

Gottron Rash / Papules

A 7 year old boy with skin scaling, erythema and edema (Gottron rash) on the extensor surface of his knees (left). After 3 days of pulse methylprednisolone therapy (right), the erythema and swelling have improved. The pigmentary changes of chronic cutaneous inflammation are more apparent after treatment.

There are Gottron papules over the metacarpophalangeal joints, proximal interphalangeal joints, and distal interphalangeal joints in this 5 year-old boy with juvenile dermatomyositis. Skin atrophy and loss of elasticity produces the papular appearance of these lesions.

Related Content

Juvenile Dermatomyositis Overview

 

Muscle Pain and/or Weakness

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Primary Care2024-05-21T13:58:22+00:00

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