Vasculitis / Immunodeficiency / Renal

Question 1

IgA Vasculitis / Henloch Schönlein Purpura

Answer 1

IgA IC’s in small vessels -> + Complement
Necrotizing (fibrinoid)
Post URTI
Palpable Purpura(lower extremities)
Arthritis
Intussusception
Renal-nephritic (IgA nephropathy)
RPGN

Question 2

Microscopic Polyangitis

Answer 2

P-ANCA (anti-myeloperoxidase)
Lungs, Kidney, Rash
Anti-GBM disease
-Type IV collagen
Alveolar BM too —> Hemoptysis (pulmonary hemorrhage)
Nephritic
Linear deposits IgG/Complement
RPGN

Question 3

RPGN (causes)

Answer 3

•Granulomatosis with Polyangitis
-C-ANCA
-Wegeners

•Microscopic Polyangitis
-P-ANCA
-Anti-GBM disease (Good Pasture)

•SLE, HSP/IgA, PSGN

Question 4

Renal Amyloidosis

Answer 4

Nephrotic Syndrome
Diffuse Mesangial staining
Congo red staining
-Apple OR green birefringence
(Seen a image that looked copper colored)

Question 5

Membranous Nephropathy

Answer 5

Nephrotic Syndrome
Granular IgG deposits and C3
Diffuse capillary wall thickening

Question 6

Wiskott Aldrich syndrome

Answer 6

B and T cell interaction problem causing deficiency with both.
Mutation in cytoskeleton MT protein

Recurrent infections that worsen with age
Triad:
•Easy bleeding (thrombocytopenia)
•Eczema
•Fair skinned
XLR

Question 7

Chediak Higashi

Answer 7

Defect in phagocyte lysosome fusion.
Oculotaneous albinism
-red eye
-silver hair
-nystagmus
Peripheral neuropathy
Giant cytoplasmic granules in neutrophils

Question 8

PSGN

Answer 8

2-4 weeks post infection
IC mediated - IgG/C3/IgM
SubEpi (lumpy bumpy/dome)
Granular

Question 9

SCIDS

Answer 9

Adenosine Deaminase deficiency
Bacterial/Viral/Candida infections in infancy
Absent thymic shadow
Absent CD3 and hypogammaglobinemia
Diarrhea
Failure to thrive

Question 10

Filtration fraction

Answer 10

FF = GFR/RPF

RPF = 1 - RBF

Question 11

CGD

Answer 11

Phagocyte metabolism disorder
No H202
Increase infection w/ catalase+ organisms

Netrophil
-NADPH Oxidase deficient (no Respiratory Burst) unable to create ROS for intracellular killing.

-Myeloperoxidase (also have)

DihydroRHODAMINE test (immunoflourescence)
= no increased fluorescence

NitroTetrazoliumBlue test too!
=does not turn blue

Question 12

Leukocyte Adhesion Deficiency

Answer 12

Defective CD18 or ICam-1 integrins
Impaired migration (chemotaxi)
-recurrent mucosal infections
-no pus
-N• leukocytosis
Infections that last too long

Question 13

X linked Agammaglobinemia

Answer 13

No IgG or T cells
All lack function
No opsonization
Tyrosine kinase defective
< 1 yr old
Recurrent sinopulmonary encapsulated bacteria infections.

Question 14

X linked Agammaglobinemia
Late onset

Answer 14

> 1 y/o
“Common variable immunodeficiency
B cell count normal- lacks function!

Question 15

Selective IgG2 deficiency

Answer 15

Runs with selective IgA deficiency
Recurrent encapsulated infections
+ = s.pneumo

• = salm, kleb, haem, pseudo, bacter, neiss, citro, b. Anthra.

Question 16

Jobs syndrome
Hyper IgE

Answer 16

Jak/stat defect
Decreased th17
Red hair
Boils and abscesses
Dermatitis
Baby teeth
Lion facies

Question 17

CREST syndrome

Answer 17

Calcinosis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodsctyly
Telangiectasias

=systemic sclerosis ( more milder type)

***anti-centromere ab

Question 18

Diffuse scleroderma

Answer 18

=systemic sclerosis
Diffuse skin and visceral involvement

**anti-DNA topoisomerase I (Scl-70)

Question 19

Anti-dsDNA

Answer 19

SLE
-malar rash
-migratory arthritis
-oral ulcers

Question 20

Anti-Histone ab’s

Answer 20

Drug induced Lupus
-procainide
-hydralazine
-Isoniazid
-D-penicillinamine
(No renal or CNS involvement)

Question 21

Anti-Ro/SSA

Anti-La/SSB

Answer 21

Sjrogen syndrome

Question 22

Anti-phospholipid ab’s

Answer 22

SLE
Anti/Phospholipid antibody syndrome
-hypercoagulable state
-miscarriages
-PTT increased

Question 23

Anti-Jo Ab’s

Answer 23

DermatoMyositis
Polymyositis
Pulmonary Fibrosis

• ab vs (histidyl-tRNA synthetase)

Question 24

Myelodysplastic Syndrome

Answer 24

Previous chemo/radiation or Old AF
- hyposegmented neutrophils
- 1 or more cytopenias

Dysplasia in bone marrow

Question 25

ThromboAngiitis Obliterans

Answer 25

Buergers
Segmental vasculitis extending into veins and nerves
Digital ischemic ulcerations

*smokers

Question 26

Temporal arteritis

Answer 26

Granulomas
Segmental
Scalp tenderness, jaw claudification
*Polymyalgia Rheumatica
-neck, torso, hip, shoulder pain
Sudden blindness

Question 27

Granulomatosis with polyangiitis

Answer 27

RPGN
C-ANCA (anti-proteinase3)
-nasal pharynx, lungs, kidneys, rash
interstitial lung disease
Granulomas via T cells
Wegeners typeIII hs

Question 28

Eosinophilic granulomatosis with polyangiitis

Answer 28

P-ANCA (anti-myeloperoxidase)
E•,IgE
Neuropathy – wrist drop foot drop
Allergy, rash