Vasculitis / Immunodeficiency / Renal Flashcards
(28 cards)
IgA Vasculitis / Henloch Schönlein Purpura
IgA IC’s in small vessels -> + Complement
Necrotizing (fibrinoid)
Post URTI
Palpable Purpura(lower extremities)
Arthritis
Intussusception
Renal-nephritic (IgA nephropathy)
RPGN
Microscopic Polyangitis
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
RPGN (causes)
•Granulomatosis with Polyangitis
-C-ANCA
-Wegeners
•Microscopic Polyangitis
-P-ANCA
-Anti-GBM disease (Good Pasture)
•SLE, HSP/IgA, PSGN
Renal Amyloidosis
Nephrotic Syndrome
Diffuse Mesangial staining
Congo red staining
-Apple OR green birefringence
(Seen a image that looked copper colored)
Membranous Nephropathy
Nephrotic Syndrome
Granular IgG deposits and C3
Diffuse capillary wall thickening
Wiskott Aldrich syndrome
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
Chediak Higashi
Defect in phagocyte lysosome fusion.
Oculotaneous albinism
-red eye
-silver hair
-nystagmus
Peripheral neuropathy
Giant cytoplasmic granules in neutrophils
PSGN
2-4 weeks post infection
IC mediated - IgG/C3/IgM
SubEpi (lumpy bumpy/dome)
Granular
SCIDS
Adenosine Deaminase deficiency
Bacterial/Viral/Candida infections in infancy
Absent thymic shadow
Absent CD3 and hypogammaglobinemia
Diarrhea
Failure to thrive
Filtration fraction
FF = GFR/RPF
RPF = 1 - RBF
CGD
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
Leukocyte Adhesion Deficiency
Defective CD18 or ICam-1 integrins
Impaired migration (chemotaxi)
-recurrent mucosal infections
-no pus
-N• leukocytosis
Infections that last too long
X linked Agammaglobinemia
No IgG or T cells
All lack function
No opsonization
Tyrosine kinase defective
< 1 yr old
Recurrent sinopulmonary encapsulated bacteria infections.
X linked Agammaglobinemia
Late onset
> 1 y/o
“Common variable immunodeficiency
B cell count normal- lacks function!
Selective IgG2 deficiency
Runs with selective IgA deficiency
Recurrent encapsulated infections
+ = s.pneumo
- = salm, kleb, haem, pseudo, bacter, neiss, citro, b. Anthra.
Jobs syndrome
Hyper IgE
Jak/stat defect
Decreased th17
Red hair
Boils and abscesses
Dermatitis
Baby teeth
Lion facies
CREST syndrome
Calcinosis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodsctyly
Telangiectasias
=systemic sclerosis ( more milder type)
***anti-centromere ab
Diffuse scleroderma
=systemic sclerosis
Diffuse skin and visceral involvement
**anti-DNA topoisomerase I (Scl-70)
Anti-dsDNA
SLE
-malar rash
-migratory arthritis
-oral ulcers
Anti-Histone ab’s
Drug induced Lupus
-procainide
-hydralazine
-Isoniazid
-D-penicillinamine
(No renal or CNS involvement)
Anti-Ro/SSA
Anti-La/SSB
Sjrogen syndrome
Anti-phospholipid ab’s
SLE
Anti/Phospholipid antibody syndrome
-hypercoagulable state
-miscarriages
-PTT increased
Anti-Jo Ab’s
DermatoMyositis
Polymyositis
Pulmonary Fibrosis
- ab vs (histidyl-tRNA synthetase)
Myelodysplastic Syndrome
Previous chemo/radiation or Old AF
- hyposegmented neutrophils
- 1 or more cytopenias
Dysplasia in bone marrow
