questions 5

Small Vessel Vasculitides with Immune Complexes

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Topic updated on 06/21/17 1:07pm

Introduction
  • Characteristics of small vessel vasculitides WITH immune complexes
    • contain immune complex deposits
  • Examples include
    • cryoglobulinemia
    • Henoch-Schonlein purpura (HSP)
    • infectious vasculitis
  • Overview of all vasculitides  
Cryoglobulinemia
  •  Overview
    • vasculitis affecting skin, GI, and renal systems
      • no joint involvement
    • relationship to HCV, type I membranoproliferative glomerulonephritis
    • antibodies that condense at decreased temperature
  • Presentation
    • Raynaud's phenomenon
    • palpable purpura
  • Epidemiology
    • adults
Henoch-Schonlein Purpura
  •  Overview
    • most common form of childhood systemic vasculitis
    • vasculitis affecting skin, GI, kidney, and joints due to IgA immune complex deposition  
      • lesions of same age
    • commonly follows URI
  • Presentation 
    • skin rash on buttocks and legs
      • palpable purpura
    • arthralgia
    • GI symptoms
      • intestinal hemorrhage
      • abdominal pain
      • melena
  • Epidemiology
    • children and young adults
  • Complications
    • Glomerulonephritis and ESRD 
    • More common in adults
Infectious Vasculitis
  •  Overview
    • vasculitis affecting skin
    • result of immune reaction to a pathogen
      • e.g., Rickettsia rickettsii (RMSF)
  • Presentation
    • RMSF involves petechiae on extremities that spreads to the trunk
  • Epidemiology
    • no age specificity


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Qbank (5 Questions)

TAG
(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? Topic Review Topic
FIGURES: A          

1. Idiopathic thrombocytopenic purpura
2. Systemic lupus erythematosus
3. Buerger's disease (thromboangiitis obliterans)
4. Berger's disease (IgA nephropathy)
5. Osler-Weber-Rendu syndrome

PREFERRED RESPONSE ▶
TAG
(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? Topic Review Topic
FIGURES: A          

1. IgA
2. IgE
3. IgG
4. C5a
5. C3b

PREFERRED RESPONSE ▶
TAG
(M1.CV.68) A three-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: Topic Review Topic
FIGURES: A          

1. Septic shock
2. Glomerulonephritis
3. Deafness
4. Rheumatic fever
5. Bowel ischemia and necrosis

PREFERRED RESPONSE ▶
TAG
(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? Topic Review Topic
FIGURES: A          

1. Vasculitis with c-ANCA
2. IgA vasculitis
3. Telescoping of bowel segment
4. Inflammatory bowel disease
5. Anti-platelet antibody

PREFERRED RESPONSE ▶
TAG
(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? Topic Review Topic
FIGURES: A          

1. Thrombocytopenia
2. Mast cell degranulation
3. Bacterial infection
4. IgA immune complex deposition
5. Venous stasis

PREFERRED RESPONSE ▶


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