Updated: 7/16/2018

Small Vessel Vasculitides without Immune Complexes

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Snapshot
  • A 34-year-old man presents to his doctor’s office for a rash of several weeks. He reports also having allergic rhinitis in the past few weeks, which was strange given that he normally has allergic rhinitis in the spring. Additionally, he reports having had childhood asthma, but in the past few months, he has had to use his inhaler for episodes of asthma. On physical exam, there is palpable purpura and urticarial plaques on the lower extremities and several skin nodules. Wheezes are heard on lung exam. Additional laboratory tests reveal peripheral eosinophilia with elevated IgE levels. (Eosinophilic granulomatosis with polyangiitis)
Introduction
  • Clinical definition
    • vasculitis mediated by neutrophils, rather than immune complexes
      • referred to as “pauci-immune” vasculitides
  • Pathogenesis
    • neutrophils directly damage vessel walls
      • ANCA antigens, proteinase 3 and myeloperoxidase, are proteins inside neutrophils that become expressed on the cell surface when activated
      • ANCA antibodies attach to these antigens and activate neutrophils, leading to vessel wall damage
    • affects small and medium-sized vessels of the skin and organs
    • cytoplasmic ANCA (c-ANCA) are directed against antigen proteinase 3 (PR3)
    • perinuclear ANCA (p-ANCA) are directed against antigen myeloperoxidase (MPO)
  • Studies
    • a biopsy may be needed to establish a diagnosis
    • direct immunofluorescence of skin biopsy is usually negative
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
  • Clinical definition
    • necrotizing granulomatous vasculitis with eosinophilia affecting multiple organs
  • Presentation
    • skin involvement
      • palpable purpura
      • urticaria
      • skin nodules
    • upper respiratory involvement
      • chronic sinusitis
      • allergic rhinitis
      • nasal polyps
    • lung involvement
      • asthma
    • neurologic involvement
      • peripheral neuropathy
        • foot or wrist drop
    • can also affect the heart, gastrointestinal system, and kidneys
  • Studies
    • histology
      • necrotizing vasculitis
      • granulomas
      • eosinophilia
    • laboratory studies
      • peripheral eosinophilia
      • ↑ IgE
      • + MPO-ANCA/p-ANCA (anti-myeloperoxidase)
  • Treatment
    • systemic glucocorticoids
    • cyclophosphamide for severe, multi-organ disease
Granulomatosis with Polyangiitis (Wegener Granulomatosis)
  • Clinical definition
    • necrotizing granulomatous c-ANCA-associated vasculitis affecting the upper respiratory tract, lungs, kidneys, and skin
  • Presentation
    • skin and mucosal involvement
      • palpable purpura and ulcers
      • friable, red gingivae
        • "strawberry gums"
      • oral ulcers
    • upper respiratory tract involvement
      • nasal ulcers
      • perforation of the nasal septum
      • chronic sinusitis, otitis media, or mastoiditis
    • lung involvement
      • hemoptysis
      • cough
      • dyspnea
    • renal involvement
      • hematuria
  • Studies and imaging
    • urine studies
      • hematuria
      • red cell casts
    • histology
      • focal necrotizing vasculitis
      • necrotizing granulomas in the respiratory tract
      • necrotizing glomerulonephritis
    • laboratory studies
      • + PR3-ANCA/c-ANCA (anti-proteinase 3)
    • chest radiography
      • large nodular densities
  • Treatment
    • cyclophosphamide
    • corticosteroids
Microscopic Polyangiitis
  • Clinical definition
    • necrotizing p-ANCA-associated vasculitis affecting the lungs, kidneys, and skin
  • Presentation
    • skin involvement
      • palpable purpura
      • ulcers
    • no upper respiratory involvement (in contrast to granulomatosis with polyangiitis)
    • pulmonary involvement
      • hemoptysis
      • dyspnea
    • renal involvement
      • hematuria
  • Studies
    • urine studies
      • hematuria
      • red cell casts
    • histology
      • necrotizing vasculitis
      • no granulomas
    • laboratory studies
      • + MPO-ANCA/p-ANCA (anti-myeloperoxidase)
  • Treatment
    • cyclophosphamide
    • corticosteroids
 

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Questions (3)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8 - 20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: <= 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, toal, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70 - 110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time (template) 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm^3
Female: 3.5-5.5 million mm^3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c <=6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm^3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm^3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm^3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm^3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m^2
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(M1.CV.80) A 21-year-old Caucasian male presents to your office with wheezing and rhinitis. Laboratory results show peripheral eosinophilia and antibodies against neutrophil myeloperoxidase. What is the most likely diagnosis? Review Topic

QID: 100596
1

Allergic bronchopulmonary aspergellosis

0%

(0/5)

2

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

80%

(4/5)

3

Cystic fibrosis

20%

(1/5)

4

Paragoniums westermani infection

0%

(0/5)

5

Pancoast tumor

0%

(0/5)

M1

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PREFERRED RESPONSE 2

(M1.CV.68) A 62-year-old Caucasian male presents to your office with hemoptysis and hematuria. On physical exam you note a saddle nose deformity. Laboratory results show an elevated level of cytoplasmic antineutrophil cytoplasmic antibody. Which of the following interventions is most appropriate for this patient? Review Topic

QID: 101022
1

Smoking cessation

4%

(1/26)

2

IV immunoglobulin

23%

(6/26)

3

Corticosteroids

54%

(14/26)

4

Isoniazid

0%

(0/26)

5

Discontinuation of ibuprofen

4%

(1/26)

M1

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PREFERRED RESPONSE 3
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