Moffitt Pearls 6.19.2017 – Rheumatology Report

Thank you to Vincent (our pulmonary fellow) for presenting a fascinating case of a young man with who was otherwise healthy presenting with muscle pain and hemoptysis found to have diffusive GGOs and cavitary nodular opacities. We discussed evaluation of hemoptysis in addition to pulmonary-renal syndromes. Final diagnosis was GPA!!

KEY PEARLS

  1. Massive hemoptysis is defined as either ≥500 mL of expectorated blood over a 24 hour period or bleeding at a rate ≥100 mL/hour.
  2. CT Approach to Hemoptysis
    1. Diffuse – corresponds with DAH
      1. Inflammatory – capillaritis
      2. Non-inflammatory or bland – cardio-pulmonary
    2. Focal
      1. Vascular: PE, AVM
      2. Nodular/Cavity lesions: Infection, Neoplasm, Rheum
      3. Mass – malignancy
      4. Septic emboli
  3. For evaluation of DAH consider CTPE as first line imaging study – You’ll get the information from the non-contrast series PLUS the addition of contrast will help r/o PE, possibly identify AVMs and provide a potential target for future interventions if needed.

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Evaluation of Hemoptysis

  • Approach to hemoptysis: airways disease (most common), pulmonary parenchymal disease, or pulmonary vascular disease, or may be idiopathic
  • CT Approach to Hemoptysis
    • Diffuse – DAH (capillaritis, vasculitis), cardio-pulmonary edema
    • Focal – Cavity, AVM, bronchiectasis,
  • Massive hemoptysis as either ≥500 mL of expectorated blood over a 24 hour period or bleeding at a rate ≥100 mL/hour.
  • History: include age, smoking history, duration and quantity of hemoptysis, and association with symptoms of acute bronchitis or an acute exacerbation of chronic bronchitis or bronchiectasis (change in sputum, blood streaking superimposed upon purulent sputum)
  • Laboratory studies to consider: Hgb, platelet count, a coagulation profile, urinalysis w/ micro, blood urea nitrogen, plasma creatinine concentration, ESR/CRP and collection of sputum for microbiologic studies.
    • ANCA, ANA, anti-GBM, complement levels
  • Imaging: Can start with CXR, but would quickly move towards CTPE – r/o PE, assess for AVMs and provides a potential target for future interventions if needed
  • DAH diagnosis: gold standard diagnosis is via BAL

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Approach to VasculitisBold face indicates can present with a pulmonary-renal syndrome

Small vessel

  • ANCA- associated or “pauci immune”
  1. GPA: c-ANCA
  2. Microscopic polyangitis: p-ANCA
  3. Eosinophilia granulomatosis with polyangitis: p-ANCA
  • Anti-GBM
  • Lupus – low C3/C4
  • Cryoglobulinemic – low C4

 

 

 

Medium vessel

  • PAN –often spares the lung
  • Kawasaki disease

Large vessel

  • Giant cell arteritis
  • Takayasu arteritis

Check out the algorithm below for an approach glomerulonephritis that highlights pulmonary-renal syndromes from Orlando Regional Medical Center:

https://www.slideshare.net/zunaira8/pulm-renal-syndromes

Picture1

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ANCA Testing

  • Immune-fluorescence testing is ideal, though in some institutions (including our own), results are reported as positivity with a secondary test for the associated antigens (as opposed to describing the location of the immune-fluorescence).
  • At Moffitt, the antigen testing comes back as a quantitative assessment of the circulating antibody to the following antigens:
    • MPO – which generally corresponds to p-ANCA antibodies
    • PR3 – which generally corresponds to c-ANCA antibodies
    • See the associated image from the Cleveland Clinic below
  • c-ANCA is associated with GPA, p-ANCA is associated with MPA and GPA, though is negative in up to 50% of cases!!
  • We don’t usually follow ANCA levels in the setting of flares as this does not correlate well with disease activity

rheumatic-fig2_large

http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/rheumatology/laboratory-evaluation-rheumatic-diseases/

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