Granulomatosis with Polyangiitis (GPA) – Recognition and Management in the ED

Hosts:
Phoebe Draper, MD
Brian Gilberti, MD

• A vasculitis affecting small blood vessels causing inflammation and necrosis
• Affects upper respiratory tract (sinusitis, otitis media, saddle nose deformity), lungs (nodules, alveolar hemorrhage), and kidneys (rapidly progressive glomerulonephritis)
• Can lead to multi-organ failure, pulmonary hemorrhage, renal failure

• Chronic sinus symptoms
• Hemoptysis (especially bright red blood)
• New pulmonary complaints
• Renal dysfunction
• Constitutional symptoms (fatigue, weight loss, fever)

• CBC, CMP for anemia and AKI
• Urinalysis with microscopy (hematuria, RBC casts)
• Chest imaging (CXR or CT for nodules, cavitary lesions)
• ANCA testing (not immediately available but important diagnostically)

• Stable patients: Outpatient workup, urgent rheumatology consult, prednisone 1 mg/kg/day
• Unstable patients: High-dose IV steroids (methylprednisolone 1 g daily x3 days), consider plasma exchange, cyclophosphamide or rituximab initiation, ICU admission

• Goodpasture syndrome (anti-GBM antibodies)
• TB, fungal infections
• Lung malignancy
• Other vasculitides (EGPA, MPA, lupus)

We discuss capacity assessment, patient autonomy, safety, and documentation.

Hosts:
Anne Levine, MD
Brian Gilberti, MD

• Arises frequently in the ED, even when not formally recognized
• Carries both legal implications and ethical weight
• Failure to appropriately assess capacity can result in:
  • Forced treatment without justification
  • Missed opportunities to respect autonomy
  • Increased risk of litigation and poor patient outcomes

• Capacity is:
  • Decision-specific: varies based on the medical choice at hand
  • Time-specific: can fluctuate due to medical conditions, intoxication, delirium
• Distinct from competency, which is a legal determination
• Relies on a patient’s ability to:
  • Understand relevant information
  • Appreciate the consequences
  • Reason through options
  • Communicate a clear choice

• Intoxicated patient with head trauma refusing CT
  • Unreliable neuro exam
  • Potentially time-sensitive intracranial injury
• Elderly patient with sepsis refusing admission due to caregiving responsibilities
  • Balancing autonomy vs. beneficence
• Patient with gangrenous diabetic foot refusing surgery
  • Demonstrates logic and consistency despite high-risk decision

• Understanding
  • Can the patient explain:
  • Their condition
  • Recommended treatments
  • Risks and benefits
  • Alternatives and outcomes?
• Sample prompts:

We dive into the recognition and management of blast crisis.

Hosts:
Sadakat Chowdhury, MD
Brian Gilberti, MD

• Blast crisis is an oncologic emergency, most commonly seen in chronic myeloid leukemia (CML).
• Defined by:
  • >20% blasts in peripheral blood or bone marrow.
  • May include extramedullary blast proliferation.
• Without treatment, median survival is only 3–6 months.

• Usually occurs in CML, but also in:
  • Myeloproliferative neoplasms (MPNs)
  • Myelodysplastic syndromes (MDS)
• Transition from chronic to blast phase often reflects disease progression or treatment resistance.

• 10% of CML patients progress to blast crisis.
• Risk increased in:
  • Patients refractory to tyrosine kinase inhibitors (e.g., imatinib).
  • Those with Philadelphia chromosome abnormalities.
  • WBC >100,000, which increases risk for leukostasis.

• Symptoms often stem from pancytopenia and leukostasis:
  • Anemia: fatigue, malaise.
  • Functional neutropenia: high WBC count, but increased infection/sepsis risk.
  • Thrombocytopenia: bleeding, bruising.
• Leukostasis/hyperviscosity effects by system:
  • Neurologic: confusion, visual changes, stroke-like symptoms.
  • Cardiopulmonary: ARDS, myocardial injury.
  • Others: priapism, limb ischemia, bowel infarction.