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From the American College of Rheumatology

Rheumatology for Primary Care
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Polyarteritis Nodosa Case Studies

Home » Polyarteritis Nodosa Case Studies
Polyarteritis Nodosa Case Studies
  • Case 1

  • Case 2

  • Case 1

A 55 yo M hx T2DM, HTN, HLD presents with unintentional weight loss. He also has abdominal pain and a new rash.

Questions to Ask

  • Other systemic symptoms?
  • Duration of symptoms?
  • Any other body systems involved?
  • Any risk factors for infection or malignancy?

History & Physical Exam

  • He is having subjective fever and night sweats.
  • On exam, he is ill-appearing and febrile (38.4C).
  • Exam shows his rash to be consistent with livedo racemosa, and there are sharply demarcated ulcerative lesions on the legs.
  • The abdominal exam is relatively unremarkable, but he has R testicular tenderness.

Diagnostic Workup

  • Initial workup:
    • CBC w diff, CMP, UA, ESR, CRP, ANCA, HIV and hepatitis serologies, blood cultures
    • Echo
    • Deep skin biopsy of an ulcerative lesion
  • To consider if Dx still unclear:
    • Testicular biopsy
    • Invasive angiography
Differential Diagnosis

Further Workup & Referrals

  • Skin involvement allows for a relatively easy biopsy target, which can be vital to diagnosis
  • Expedited referral to rheumatology for high dose IV corticosteroids

Treatment & Management

  • Treatment would likely involve high-dose steroids and cyclophosphamide (under rheumatology care)
  • Monitor glucose, blood pressure, blood counts, UA (hemorrhagic cystitis risk), evidence of infection, secondary malignancy
  • Case 2

A 59 yo female hx obesity and iron-deficiency anemia presents with transient monocular vision loss, as well as subacute fatigue and malaise.

Questions to Ask

  • Any other eye symptoms?
  • Duration of symptoms?
  • Any other body systems involved?
  • Traditional cardiovascular risk factors?
  • Cardioembolic risk factors?

History & Physical Exam

  • 2 episodes of acute R eye complete vision loss that lasted 3-5 min before resolving
  • No current headache, diplopia or jaw claudication
  • Vision and external eye exams are normal.
  • She also developed L wrist weakness suddenly 2 weeks ago, and both a L wrist extension and R dorsiflexion weakness are noted on exam.
  • Cardiac, pulmonary, skin, HEENT and joint exams are normal.

Diagnostic Workup

  • Initial workup:
    • CBC w diff, CMP, ESR, CRP, ANCA, HIV, HBV, blood cultures
    • Echo
    • EMG
  • Consider:
    • Sural nerve biopsy
    • Temporal artery biopsy
Differential Diagnosis

Further Workup & Next Steps

  • This severe presentation warrants hospitalization and empiric therapy (steroids).
  • Initial workup should prioritize evaluation for systemic inflammation and characterization of her neurologic deficits.
  • GCA is the most likely vasculitis to cause amaurosis, but with MM, PAN or ANCA-associated vasculitis would be more likely.
  • ANCA serologies and biopsy would help clarify the diagnosis.

Differential Diagnosis

His systemic symptoms are non-specific.

Consider DDx: infection, cancer, etc.

Differential Diagnosis

Her history is consistent with amaurosis fugax, which suggests a vascular cause.

  • Amaurosis is not common in PAN (more common in GCA) but can occur.
  • Her pattern of acute asymmetric weakness is characteristic of mononeuritis multiplex (MM), which strongly suggests a systemic vasculitis with medium-sized vessel (vasa nervorum) involvement.
View Information About Polyarteritis Nodosa
Tami Bonnett-Admi2024-02-16T03:51:35+00:00

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