Skip to content

From the American College of Rheumatology

Rheumatology for Primary Care
  • Symptoms
    • Fever
    • Joint Pain
    • Muscle Pain and/or Weakness
    • Rashes
    • Raynaud’s Phenomenon
  • Diseases
    • ANCA-Associated Vasculitis
    • Behcet’s Syndrome
    • Crystalline Arthropathies
    • Drug and Medication-Induced Rheumatic Diseases
    • Giant Cell Arteritis
    • Henoch-Schönlein Purpura
    • IgG4-Related Disease
    • Juvenile Dermatomyositis
    • Juvenile Idiopathic Arthritis
    • Kawasaki Disease
    • Mixed Connective Tissue Disease
    • Multisystem Inflammatory Syndrome in Children
    • Myositis
    • Polyarteritis Nodosa
    • Polymyalgia Rheumatica
    • Rheumatoid Arthritis
    • Sarcoidosis
    • Sjogren’s Disease
    • Spondyloarthritis
    • Systemic Lupus Erythematosus
    • Systemic Sclerosis
  • Case Studies
    • ANCA-Associated Vasculitis
    • Behcet’s Syndrome
    • Crystalline Arthropathies
    • Giant Cell Arteritis
    • Henoch-Schönlein Purpura
    • IgG4-Related Disease
    • Juvenile Dermatomyositis
    • Juvenile Inflammatory Arthritis
    • Kawasaki Disease
    • Mixed Connective Tissue Disease
    • Myositis
    • Pediatric Fevers
    • Polyarteritis Nodosa
    • Polymalgia Rheumatica
    • Raynaud’s Phenomenon
    • Rheumatoid Arthritis
    • Sarcoidosis
    • Sicca/Sjogren’s Disease
    • Spondyloarthritis
    • Systemic Lupus Erythematosus
    • Systemic Sclerosis
  • Labs
  • Meds
    • Commonly Used Rheumatology Meds
    • Treatment Considerations
    • Addressing Patient Fears About Rheumatology Medications
    • Medications by Diseases
  • Resources
    • Resources
    • Vaccination Considerations – Pediatrics
    • Vaccination Considerations – Adults
    • Glossary of Terms
    • ACR Clinical Guidelines
    • ACR Education Center
    • Rheumatology Provider Directory

Polyarteritis Nodosa Case Studies

Home ยป Polyarteritis Nodosa Case Studies
Polyarteritis Nodosa Case Studies
  • Case 1

  • Case 2

  • Case 1

A 55 yo M with history of T2DM, HTN, and 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.
  • 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:
    • Invasive angiography
Differential Diagnosis

Further Workup & Referrals

  • Full thickness biopsy of ulcer looking for medium vessel vasculitis
  • Expedited referral to rheumatology for high-dose IV corticosteroids

Treatment & Management

  • Treatment with high-dose steroids +/- cyclophosphamide depending on imaging findings and presence of organ threatening disease (under rheumatology care)
  • Monitor glucose, blood pressure, blood counts, UA (hemorrhagic cystitis risk), evidence of infection, secondary malignancy
  • Case 2

A 59 yo female with history of 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 can happen with PAN
  • If there is mononeuritis multiplex (foot drop, hand drop), PAN or ANCA-associated vasculitis would be more likely.
  • Deep biopsy is helpful to identify if this is medium vessel vasculitis.

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.

Related Content

Polyarteritis Nodosa Overview

 

Giant Cell Arteritis Overview

 

ACR Vasculitis Treatment Guideline

We Want Your Input

Find what you needed? We would love your feedback so that we can ensure the site meets the needs of primary care providers.

Give Feedback
Primary Care2024-05-21T13:47:32+00:00

American College of Rheumatology Information

About ACR

ACR Contacts

Authors

This product was funded by a medical grant from Pfizer. The content was written by a physician work group. See Authors

© Copyright American College of Rheumatology 2025 | All rights reserved | See ACR Policies

Page load link
Go to Top