Updated: 11/26/2019

Granulomatosis with Polyangiitis

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Snapshot
  • A 55-year-old man presents to the emergency department due to coughing up blood in the morning. His symptoms are accompanied by fever, arthralgias affecting the wrists and knees, and unintentional weight loss over the course of 3 months prior to presentation. The patient also noticed mild shortness of breath. On physical exam, there is mild nasal crusting and bloody nasal discharge. There are no lesions in the mouth. There is tenderness to palpation of the wrist and knees. Laboratory testing is significant for an elevated erythrocyte sedimentation rate, C-reactive protein, and a creatinine of 2.5 mg/dL (his creatinine is normally 0.9 mg/dL). A radiograph of the chest demonstrates pulmonary nodules. 
Introduction
  • Clinical definition
    • small- to medium-sized vessel vasculitis and granulomatosis that affects
      • upper and lower respiratory tract and kidneys 
    • previously known as Wegener granulomatosis
  • Epidemiology
    • incidence
      • can occur at any age but typically between 65-75 years of age
  • Etiology
    • unclear but may involve both genetic and environmental components
  • Pathogenesis
    • antiproteinase-3 (PR3 ANCA/c-ANCA) antibodies present in ~95% of cases 
    • T-cell mediated hypersensitivity reaction that leads to granuloma formation
  • Associated conditions
    • rapidly progressive glomerulonephritis
  • Prognosis
    • glucocorticoids can lead to remission at 6 months in > 90% of patients
Presentation
  • Symptom/physical exam  
    • upper respiratory symptoms
      • sinusitis (most common)
      • recurrent otitis media
      • otalgia
    • lower respiratory symptoms
      • hemoptysis
    • renal symptoms
      • hematuria
    • skin
      • leukocytoclastic angiitis
Imaging
  • Radiography
    • indication
      • should be performed in all patients with pulmonary involvement likely secondary to ANCA-associated vasculitis
        • computerized tomography scan is also performed
    • views
      • chest
    • findings
      • single or multiple nodules/masses in ~66% of cases
      • nodules may be cavitated
Studies
  • Labs
    • ↑ C-reactive protein and erythrocyte sedimentation rate
    • positive PR3 ANCA/c-ANCA testing  
  • Biopsy the site of active disease
    • confirms the diagnosis
    • demonstrates granulomas, giant cells, necrosis, and vasculitis
Differential
  • Microscopic polyangiitis
    • differentiating factor
      • does not present with nasopharyngeal involvement, which is seen in granulomatosis with polyangiitis
      • no granuloma formation
      • MPO-ANCA/p-ANCA positive
  • Polyarteritis nodosa
    • differentiating factor
      • renal infarcts, artery stenosis, and visceral microaneurysms are classic for polyarteritis nodosa
      • not associated with ANCA antibodies
Treatment
  • Medical 
    • high-dose glucocorticoids 
      • indication
        • initial treatment option in patients with non-life or organ-threatening disease
          • typically given with methotrexate
    • high-dose glucocorticoids with cyclophosphamide or rituximab
      • indication
        • initial treatment option in patients with life- or organ-threatening disease
Complications
  • Pulmonary hemorrhage
  • Respiratory failure
  • Uremia
  • Renal failure
  • Palpable purpura and other vascular lesions
 
 

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Questions (3)
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(M2.RH.17.4769) A 45-year-old male presents to the emergency room complaining that he has been coughing up blood-tinged sputum. Since this morning, the patient reports he has coughed up around 1/4 cup of "phlegm" mixed with blood. A review of systems is also notable for fatigue and night sweats that began two nights ago, but he denies any recent changes in weight. His past medical history is significant for hypertension, chronic sinusitis, and childhood asthma. His current medications include metoprolol and amlodipine. He is a smoker with a 20 pack-year history. The patient’s temperature is 99°F (37.2°C), blood pressure is 126/76 mmHg, pulse is 72/min, and respirations are 14/min with an oxygen saturation of 98% O2 on room air. A physical exam is notable for oral ulcers. A chest radiograph is performed and is shown in Figure A. Labs are drawn and are listed below:

Serum:
Na+: 144 mEq/L
Cl-: 109 mEq/L
K+: 4.3 mEq/L
HCO3-: 23 mEq/L
Urea nitrogen: 46 mg/dL
Glucose: 100 mg/dL
Creatinine: 3.0 mg/dL

Leukocyte count and differential:
Leukocyte count: 12,000/mm^3
Segmented neutrophils: 60%
Bands: 3%
Eosinophils: 3%
Basophils: < 1%
Lymphocytes: 28%
Monocytes: 5%
Hemoglobin: 13.0 g/dL
Hematocrit: 50%
Platelets: 200,000/mm^3

Urine:
Epithelial cells: 5/hpf
Glucose: negative
Proteins: 25 mg/dL
RBC: 15/hpf
Red cell casts: 7/hpf
WBC: 1/hpf
Leukocyte esterases: negative
Nitrites: negative
Bacteria: none

Based on the information above, which of the following is most likely to be abnormal?
Tested Concept

QID: 109138
FIGURES:
1

Sputum acid-fast stain

9%

(3/35)

2

Test for anti-myeloperoxidase

17%

(6/35)

3

Test for anti-proteinase 3

54%

(19/35)

4

Renal arteriogram

11%

(4/35)

5

Sputum cytology

6%

(2/35)

M 6 D

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(M2.RH.15.4672) A 42-year-old female reports progressively worsening dyspnea and cough for the past year. She also complains of concurrent joint pains in her bilateral knees, elbows, and wrists. Upon further history, the patient notes that she has “really bad allergies”, with symptoms of nasal congestion, sinus pressure, and nosebleeds a few times each month. Urinalysis is positive for 1+ blood and 2+ protein. Her creatinine is elevated at 1.3 mg/dL. Physical exam reveals the following in her lower extremities (Figure A). Biopsy of these lesions yields the following findings (Figure B). What is the most likely positive serologic finding in this patient? Tested Concept

QID: 107185
FIGURES:
1

Antineutrophil cytoplasmic antibodies recognizing myeloperoxidase

35%

(9/26)

2

Antineutrophil cytoplasmic antibodies recognizing proteinase 3

58%

(15/26)

3

Autoantibodies recognizing cyclic citrullinated peptide

0%

(0/26)

4

Autoantibodies recognizing nuclear RNA-binding proteins

4%

(1/26)

5

Antibodies that precipitate and aggregate upon exposure to cold temperature

0%

(0/26)

M 6 E

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