Clinical Features

  • Insidious onset (weeks in HIV+) vs rapid/fulminant onset (days in non-HIV).
  • Classic triad:
    • Progressive exertional dyspnea
    • Dry/nonproductive cough
    • Fever
  • Profound hypoxemia disproportionate to physical exam findings (pulmonary auscultation often normal or shows minimal faint crackles). c

Diagnosis

  • Initial Lab Testing:
    • Serum LDH: Elevated (> 220 U/L) in > 90% of cases (high sensitivity, low specificity).
    • ABG: Hypoxemia, ↑ A-a oxygen gradient (≥ 35 mmHg).
    • Serum (1,3)-β-D-glucan: High sensitivity; supports diagnosis if positive.
  • CXR/CT: Bilateral, diffuse “ground-glass” opacities.
  • Definitive Dx: Organisms seen on bronchoalveolar lavage (BAL) sample.
  • Stains: Methenamine silver stain shows disc-shaped or “crushed ping-pong ball” cysts.

Management

  • Emergency & Oxygenation:
    • Supplemental O₂ to maintain SaO₂ > 92%.
  • Adjuvant Corticosteroids (CRITICAL HIGH-YIELD STEP): c
    • Indication: PaO₂ < 70 mmHg OR A-a gradient ≥ 35 mmHg on room air.
    • Timing: Administer prior to or concurrently with antibiotic therapy (prevents inflammatory flare from fungal lysis).
    • Regimen: IV Methylprednisolone or PO Prednisone tapered over 21 days.
  • First-Line Treatment:
    • TMP-SMX (IV or PO high-dose) x 21 days.
  • Alternative Treatment (Sulfa Allergy / Severe Intolerance):
    • Mild-to-Moderate disease: Atovaquone OR TMP + Dapsone.
    • Moderate-to-Severe disease: IV Pentamidine OR Clindamycin + Primaquine.
  • Prophylaxis (Prevention):
    • Indications:
      • HIV w/ CD4 < 200 cells/mm³, CD4 percentage < 14%, or history of oral candidiasis.
      • Solid organ transplant / chronic high-dose steroid therapy.
    • First-lineTMP-SMX (1 DS or 1 SS tablet daily).
    • Alternatives: Dapsone, Atovaquone, or Aerosolized Pentamidine.
    • Discontinue in HIV when CD4 > 200 cells/mm³ for > 3 months on ART.