Updated: 3/31/2018

Small Vessel Vasculitides with Immune Complexes

Topic
Review Topic
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Questions
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Evidence
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Snapshot
  • A 5-year-old boy presents to the emergency room for abdominal pain and rash on his legs. He also reports having intermittent joint pains. A few weeks ago, he had an upper respiratory infection. On physical exam, he has nontender palpable purpura on his buttocks and lower extremities. His abdomen is mildly tender. Urine studies show proteinuria and hematuria. (Henoch-Schonlein purpura/IgA vasculitis)
Introduction
  • Clinical definition
    • vasculitis caused by immune complex deposition
  • Pathogenesis
    • circulating antigens induce antibody formation
      • the antibodies then bind to antigens, creating immune complexes
    • the immune complexes then deposit in small vessels, activating complement and causing a vasculitis
  • General clinical features
    • palpable dark-red or purple purpura
    • may itch, burn, or be nontender
    • may ulcerate
  • Studies
    • most cases are diagnosed clinically, but a biopsy may be needed
    • direct immunofluorescence
      • deposition of C3, IgM, IgA, and/or IgG
Cryoglobulinemic Vasculitis
  • Clinical definition
    • small-vessel vasculitis caused by mixed cryoglobulins affecting the skin, peripheral nervous system, and kidneys
      • associated with hepatitis C virus (HCV) infection
      • occurs in adults
  • Presentation
    • peripheral neuropathy
    • skin involvement
      • palpable purpura
      • ulcerations
      • livedo reticularis
    • joint pain
    • renal involvement
      • membranoproliferative glomerulonephritis
    • no gastrointestinal symptoms (in contrast to Henoch-Schonlein purpura)
  • Studies
    • cryoglobulins isolated from the serum
    • urine studies
      • proteinuria
      • hematuria
  • Treatment
    • treat underlying HCV infection
    • corticosteroids
Henoch-Schonlein purpura (IgA Vasculitis)
  • Clinical definition
    • IgA-mediated small vessel vasculitis that often follows an upper respiratory infection  
      • especially with group A streptococcus or parvovirus B19
      • occurs in children
  • Presentation
    • triad of 
      • nontender palpable purpura
        • on buttocks and lower extremities
        • may ulcerate
      • arthralgias
      • colicky abdominal pain
    • constitutional symptoms
    • hematuria
  • Studies
    • urine studies
      • proteinuria
      • hematuria
    • direct immunofluorescence
      • IgA deposition in small vessels on direct immunofluorescence
    • histology
      • IgA deposition in small vessels on staining
      • leukocytoclasis
  • Treatment
    • typically resolves spontaneously over time
    • angiotensin-converting enzyme (ACE) inhibitor
      • indication
        • for persistent proteinuria, which may result in end-stage renal disease or glomerulonephritis
 

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Questions (5)
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, total, 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 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
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/mm3
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 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
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/mm3
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/m2
Calculator

(M1.CV.68) A 3-year-old female presents to the emergency department with colicky abdominal pain, polyarthralgias and the palpable rash shown in Figure A. You are concerned that complications of her disease may include: Review Topic

QID: 100584
FIGURES:
1

Septic shock

0%

(0/4)

2

Glomerulonephritis

75%

(3/4)

3

Deafness

0%

(0/4)

4

Rheumatic fever

0%

(0/4)

5

Aortic regurgitation

25%

(1/4)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(M1.CV.74) A 7-year-old boy is brought to the emergency department by his mother. He had a recent cold, and is now complaining of colicky abdominal pain. However, his mother became very concerned and brought him to the emergency department when she noted the rash shown in Figure A. On further review, the boy says that his elbows and knees have been hurting him. A complete blood count is within normal limits. What type of pathological process underlies his disorder? Review Topic

QID: 106683
FIGURES:
1

Vasculitis with c-ANCA

17%

(17/102)

2

IgA vasculitis

69%

(70/102)

3

Telescoping of bowel segment

3%

(3/102)

4

Inflammatory bowel disease

2%

(2/102)

5

Anti-platelet antibody

8%

(8/102)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(M1.CV.4732) An 8-year-old girl is brought to the physician by her parents due to a new rash on her feet and legs. Her parents noticed the rash one day ago, and the child has also been complaining of joint pain and abdominal pain. She has no chronic medical conditions and has not been traveling or spending time outdoors recently. She had an upper respiratory infection seven days ago which resolved on its own. On exam, her vital signs are normal, and she is generally well-appearing. Her joints have full range of motion with no evidence of trauma. Her abdomen is soft and non-distended and is mildly tender to deep palpation diffusely. The patient has the notable findings in Figure A which are non-blanchable. The results of a complete blood count are within normal limits. Her urinalysis shows > 20 RBCs.

What is most likely responsible for this patient’s dermatologic findings? Review Topic

QID: 108620
FIGURES:
1

Thrombocytopenia

23%

(46/196)

2

Mast cell degranulation

3%

(5/196)

3

Bacterial infection

8%

(15/196)

4

IgA immune complex deposition

63%

(124/196)

5

Venous stasis

0%

(0/196)

M1

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PREFERRED RESPONSE 4

(M1.CV.47) An 3-year-old male complains of abdominal pain following an upper respiratory infection. On physical examination, you note the dermatologic abnormality shown in Figure A. Which of the following is most likely involved in the pathogenesis of this patient's disease? Review Topic

QID: 100468
FIGURES:
1

IgA

61%

(57/93)

2

IgE

12%

(11/93)

3

IgG

8%

(7/93)

4

C5a

8%

(7/93)

5

C3b

12%

(11/93)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 1

(M1.CV.2) An 8-year-old boy presents to your office with joint pain, hematuria, and blood in the stool following an episode of pharyngitis 2 weeks ago. Examination reveals palpable red lesions on the lower extremities as seen in Figure A. This patient's disease is most similar to which of the following diseases? Review Topic

QID: 100956
FIGURES:
1

Idiopathic thrombocytopenic purpura

20%

(1/5)

2

Systemic lupus erythematosus

0%

(0/5)

3

Buerger's disease (thromboangiitis obliterans)

0%

(0/5)

4

Berger's disease (IgA nephropathy)

80%

(4/5)

5

Osler-Weber-Rendu syndrome

0%

(0/5)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4
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