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From the American College of Rheumatology

Rheumatology for Primary Care
  • Symptoms
    • Fever
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    • Raynaud’s Phenomenon
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    • ANCA-Associated Vasculitis
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  • Case Studies
    • ANCA-Associated Vasculitis
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    • Giant Cell Arteritis
    • Henoch-Schönlein Purpura
    • IgG4-Related Disease
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    • Mixed Connective Tissue Disease
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    • Polymalgia Rheumatica
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ANCA-Associated Vasculitis Case Studies

Home ยป ANCA-Associated Vasculitis Case Studies
ANCA-Associated Vasculitis Case Studies
  • Case 1

  • Case 2

  • Case 1

A 66 yo M with no past medical history presents with hemoptysis. Patient 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, RBCs = ~20 per hpf
  • ANCA with positive C-ANCA 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
  • Case 2

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: sinusitis?
  • Cardiac: new-onset chest pain?
  • Cutaneous: history of eczema?

Physical Exam

  • Vitals stable
  • Neurologic: 2/5 strength left foot 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 cyclophosphamide

Diagnosis

Patient has eosinophilic granulomatosis with polyangiitis (eGPA).

Related Links

References

 

ANCA-Associated Vasculitis Overview

 

ACR Vasculitis Treatment Guideline

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Primary Care2024-05-21T13:42:36+00:00

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