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.
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CME / ABIM MOC Released: 11/7/2022
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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.
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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 |
|
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 | ||
Creatinine, mg/dL | 3.6 | Male: 0.7 to 1.3 Female: 0.6 to 1.1 |
CRP, mg/dL | 21.5 | < 1.0 |