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Case Challenges in Small-Vessel Vasculitis: Can You Diagnose and Manage These Patients?

  • Authors: Kenneth J. Warrington, MD
  • CME / ABIM MOC Released: 11/7/2022
  • Valid for credit through: 11/7/2023, 11:59 PM EST
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Target Audience and Goal Statement

This activity is intended for rheumatologists, nephrologists, pulmonologists, and other healthcare professionals who provide care to patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV).

The goal of this activity is for learners to be better able to recognize, diagnose, and treat various subtypes of AAV.

Upon completion of this activity, participants will:

  • Have greater competence related to
    • Differentiating the subtypes of AAV
    • Selecting induction therapy to achieve remission in patients with AAV
    • Selecting therapy to reduce risk of glucocorticoid toxicity in patients with AAV
  • Have increased confidence related to
    • Monitoring patients with AAV for relapse


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  • Kenneth J. Warrington, MD

    Professor of Medicine
    Mayo Clinic
    Rochester, Minnesota 


    Kenneth J. Warrington, MD, has the following relevant financial relationships: 
    Consultant or advisor for: ChemoCentryx
    Research funding from: Eli Lilly; GlaxoSmithKline; Kiniksa 


  • Ashley Stumvoll, MRes

    Associate Medical Writer, Medscape, LLC


    Ashley Stumvoll, MRes, has no relevant financial relationships.

Compliance Reviewer

  • Leigh Schmidt, MSN, RN, CNE, CHCP

    Associate Director, Accreditation and Compliance, Medscape, LLC


    Leigh Schmidt, MSN, RN, CNE, CHCP, has no relevant financial relationships.

Peer Reviewer

This activity has been peer reviewed and the reviewer has no relevant financial relationships.

Accreditation Statements

Developed through a partnership between Medscape and American College of Rheumatology.


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Case Challenges in Small-Vessel Vasculitis: Can You Diagnose and Manage These Patients?

Authors: Kenneth J. Warrington, MDFaculty and Disclosures

CME / ABIM MOC Released: 11/7/2022

Valid for credit through: 11/7/2023, 11:59 PM EST


The following cases are modeled on the interactive grand rounds approach. The questions within the activity are designed to test your current knowledge. After each question, you will be able to see whether you answered correctly and read evidence-based information that supports the most appropriate answer choice. The questions are designed to challenge you; you will not be penalized for answering the questions incorrectly. At the end of the activity, there will be a short post-test assessment based on the material presented.


Damien, a 67-year-old White man, presents to the emergency department (ED) complaining of malaise, rash, difficulty breathing, and coughing up blood. He has type 2 diabetes and a history of hypertension. He reports that his symptoms started approximately 2 months ago but have rapidly escalated within the past week. The ED staff ask whether he has previously sought medical treatment for his symptoms, and Damien states that he visited his primary care practitioner before his sputum became bloody and was prescribed an albuterol inhaler for his pulmonary symptoms. He was also advised to treat the rash with over-the-counter topical glucocorticoids. Damien is seen in the ED, and the physician orders laboratory studies and a chest x-ray.

Based on clinical evaluation and initial diagnostic studies, Damien is admitted to the hospital. Suspecting that Damien is displaying symptoms of vasculitis, rheumatology is called in to consult. As the rheumatologist on call, you go to examine the patient and review the lab results.

Physical Presentation and Imaging (Table 1)

When you enter the treatment room, you notice that Damien does not immediately respond to your presence and appears lethargic. He presents with an elevated heart rate (100 beats per minute) and a mild fever (100.5 °F). Despite an elevated heart rate, Damien's heart seems otherwise normal. His ears, nose, and throat appear normal. He does not appear to have any musculoskeletal issues and does not report pain upon palpation of his abdomen. The skin on Damien's lower extremities is covered in a diffuse, purpuric rash. Upon listening to Damien's lungs, you identify the presence of inspiratory crackles, which appear to be bilateral. The x-rays ordered at intake by ED staff reveal presence of bilateral alveolar infiltrates.

Laboratory Results (Table 2)

When the complete blood cell (CBC) count with differential results returns, you notice that Damien's white blood cell count and neutrophil levels are elevated. His red blood cell count (2.8 million/µL), hemoglobin (8.5 g/dL), and hematocrit (27.7%) levels are all low. The creatinine test reveals significantly elevated creatinine (3.6 mg/dL). The C-reactive protein (CRP) test also shows extremely elevated levels of CRP (21.5 mg/dL).

Damien's health information and test result charts along with reference ranges for relevant lab metrics are shown in Tables 1 to 3.

Table 1. Patient History

History Findings
Medical Type 2 diabetes mellitus, hypertension
Current medications Albuterol, topical glucocorticoids
Allergies None
Psychosocial Anxiety associated with unexplained symptoms
Immunizations All immunizations are up to date, eligible for additional mRNA COVID-19 booster

mRNA, messenger RNA.

Table 2. Physical Examination and Imaging

Physical Examination Findings
Vital signs
  • Height = 5'9"
  • Weight = 155 lb
  • BMI = 23 kg/m2
  • BP = 140/90 mm Hg
  • Heart rate = 110 bpm
  • Temperature: 100.5 °F
General Appears lethargic
Skin Diffuse, purpuric rash over both lower extremities
Chest and lungs Inspiratory crackles bilaterally
Chest x-ray showed bilateral alveolar infiltrates
Heart Normal
Abdomen Normal
ENT Normal
MSK Normal

BMI, body mass index; BP, blood pressure; bpm, beats per minute; ENT, ear, nose, and throat; MSK, musculoskeletal.

Table 3. Recent Relevant Lab Work: Tests Ordered by ER at Intake

Tests Result Reference Range
CBC with differential/platelet
White blood cell count, cells/µL 13,600 4500 to 10,000
Lymphocytes, absolute, cells/µL 850 800 to 5000
Monocytes, absolute, cells/µL 500 400 to 1000
Neutrophils, absolute, cells/µL 12,100 1800 to 8300
Eosinophils, absolute, cells/µL 100 0 to 800
Basophils, absolute, cells/µL 20 0 to 100
Red blood cell count, million/µL 2.8 Male: 4.7 to 6.1
Female: 4.2 to 5.4
Reticulocytes, % 2.93 0.5 to 2.0
Hemoglobin, g/dL 8.5 Male: 13.8 to 17.2
Female: 12.1 to 15.1
Hematocrit, % 27.7 Male: 40.7 to 50.3
Female: 36.1 to 44.3
Mean corpuscular volume, fL 96 80 to 95
Red cell distribution width, % 14.5 11 to 15
Platelet count, per µL 465,000 150,000 to 400,000
Creatinine, mg/dL 3.6 Male: 0.7 to 1.3
Female: 0.6 to 1.1
CRP, mg/dL 21.5 < 1.0
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