Case 34--Rapidly Progressive Respiratory Failure in a Woman with Features of Dermatomyositis

Clinical summary: A 64-year-old white woman was transferred to UCSF on 6/5 with respiratory failure. Following a strenuous trip to Italy 4 weeks before admission, she developed increasing fatigue, anorexia, and weight loss, but no fever or chills. Two weeks later, increasing dyspnea, pleurisy, and chest tightness prompted admission to another hospital. There was no history of exposure to toxins, pets, animals, or moldy hay. She did not use tobacco or alcohol. She was a thin woman with a reddish rash on the cheeks, eyelids, and forehead. Fine rales were heard over the chest. A chest radiograph showed bilateral, basilar opacities, suggesting interstitial pneumonia. The sedimentation rate was 44 mm/hr. HRCT and PFTs were performed. Bronchoscopy yielded no pathogens. A transbronchial biopsy was performed. Solumedrol 60 mg/d was begun on 6/4, and she was transferred to UCSF. On admission to UCSF, vital signs were: heart rate 90/min, blood pressure 120/80, temperature 36.5 degrees, respiratory rate 25/minute. The oxygen saturation was 90% on 100% oxygen. Physical examination showed a macular rash on the face (as above) and erythema of the neck and upper chest. A scaling rash was present over the lateral surfaces of the fingers (mechanic's hands), and there was a papulosquamous rash on both elbows. Laboratory tests showed a hemoglobin of 11.3 g/dl and a WBC of 11.5 k/µl, rising to 25.3 k/µl 4 days later. A biopsy of the skin of the forehead was taken. Despite increasing the solumedrol to 1000 mg/da on admission, respiratory failure progressed, and mechanical ventilation was begun. Cyclophosphamide was begun on 6/8. She expired on 6/10. An autopsy was limited to the lungs.

Laboratory values

Serologies

  • ANA +, 1:80, speckled
  • Anti-double stranded DNA 6 (normal <30)
  • Anti-Jo-1 antibody negative
  • C- and p-ANCA negative

Muscle enzymes

  • Creatine phosphokinase 22 (normal 39-189)
  • Aldolase 8 (normal 2-6.5)

Liver enzymes

ALT and AST are also muscle enzymes

  • Alanine aminotransaminase 65 (normal 11-54)
  • Aspartate aminotransaminase 94 (normal 16-41)
  • Total bilirubin 0.4
  • Alkaline phosphatase 86 (normal 29-111)

Arterial blood gases

  • 6/5 pH 7.45, pCO2 41, pO2 78, on 100% O2
  • 6/9 pH 7.13, pCO2 62.6, pO2 60 on 100% O2

Which features suggest the diagnosis of dermatomyositis? Answer

Radiology | Image 1 | Image 2 | Image 3 | Image 4 | Image 5 | Image 6 | Discussion

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Answer--Features of dermatomyositis are: