A 66 yo M with no pmhx presents with hemoptysis. Pt has significant weight loss, fevers, and history of chronic sinusitis.

Questions to Ask

  • Onset: Gradual vs acute? Sick exposures?
  • Constitutional: Fevers, weight loss, fatigue?
  • ENT: Sinusitis, septal deformities, epistaxis, hearing loss?
  • Nervous: Mononeuritis multiplex (foot drop)?
  • Cutaneous: Purpura, ulcers?

Physical Exam

  • Diffuse rales on pulmonary exam
  • Vitals stable
  • Otherwise normal exam

Diagnostic Workup

Labs
  • CBC with Hgb 9 (no prior), CMP with Cr 1.9, UA 2+ blood, TMTC RBCs
  • ANCA with positive cANCA and PR3 antibodies
Further Workup
  • Kidney bx: pauci-immune crescentic glomerulonephritis
  • Bronch: consistent with DAH

Treatment

  • Start pulse steroids

Referrals

  • Consult rheumatology for consideration of rituximab vs. cyclophosphamide

A 54 yo F presents with foot drop. Further hx reveals longstanding hx asthma, fatigue, weight loss, otherwise negative.

Questions to Ask

  • Onset: Gradual vs. acute? Sick exposures?
  • Constitutional: Fevers, weight loss, fatigue?
  • Respiratory: Shortness of breath?
  • ENT: Hx chronic sinusitis?
  • Cardiac: New-onset chest pain?
  • Cutaneous: Hx eczema?

Physical Exam

  • Vitals stable
  • Neurologic: 2/5 strength left dorsiflexion
  • Respiratory: lungs clear

Diagnostic Workup

Labs
  • ANCA negative
  • CBC: absolute eosinophil count 2300 (20% on differential), CMP unremarkable
Further Workup
  • Sural nerve bx: Necrotizing vasculitis
Diagnosis

Treatment

  • Pulse dose steroids for severe eosinophilic granulomatosis with polyangiitis (eGPA) (neurologic involvement)

Referrals

  • Consult rheumatology for initiation of cyclophophamide