Lupus and Sjogrens Flashcards
Systemic Lupus Erythematosus definition?
Multi-system inflammatory disorder
Autoantibodies to numerous self antigens
Discoid lupus
only involves the skin though these Discoid lesions CAN be seen in SLE
Drug induced Lupus
Less severe than SLE
Self-limiting once the offending drug is removed.
Neonatal lupus
Newborns of mothers with SLE
-skin rash, heart block (permanant), thrombocytopenia
What are the three problems of neonatal lupus
skin rash, heart block(which is permanent), and thrombocytopenia
Female to male ration in lupus
13:1
Peak incidence of lupus?
Childbearing years
Racial discrimination
Blacks 3-4 times more likely
Lupus is called the disease of a thousand faces. WHy
Can present in tons of different ways
Common general symptoms of lupus
Malaise, fever, fatigue
Cutaneous manifestations of lupus
Malar rash, alopecia
Malar rash, along with other symptoms, get worse upon exposure to what?
SUN
Discoid lesions heal with…..
scars
Musculoskeletal complaints
Arthralgias, arthritis, myalgias, myositis
Arthralgia and myalgia are just joint and muscle pain. The -itis’ are inflammation
Ulnar deviation that can be corrected occurs in what dz?
Lupus
Ulnar deviation that cannot be corrected (fixed ulnar deviation) occurs in
Rheumatoid Arthritis
DrP Key: what kind of genetic deficiency confers the highest risk of dz
Complement Deficiency
DrP Key: This mutation is important in lupus and it points to impaired regulation of endogenous nucleic acids
TREX1
DrP Key: The production and affect of …… has emerged as central to Lupus Pathogenesis
Type 1 Interferon
Renal Disorders in lupus are correlated with the presence of what?
anti-dsDNA antibodies
If renal dz is active the anti-dsDNA is high
What is the most common renal structure affected
Glomerulus….they all CAN be affected.
- – RBC casts in Urine
- – And proteinuria…….this is a glomerulonephritis, dawg
Psychiatric abnormalities in Lupus?
Depression, psychosis, cognitive abnormalities
Neurological dz
STROKE, myelitis,
What do you first do if a pt presents with neurological disease like a seizure and they have lupus?
EXCLUDE OTHER CAUSES like a brain tumor
Heme manifestation
Anemia, thrombocytopenia, leukopenia
Most common pulmonary maifestation
pleuritis with pleural effusion
Most common cardiac manifestation
pericarditis
What can happen on the valves
Libman sacks endocarditis
Obstetrical complications of Lupus?
Small babies, and receurrent fetal loss
Anticardiolipin/ antiphospholipid antibody syndrome can cause what?
blood clots, can lead to multiple miscarriages.
Lupus classification mnemonis
RASH PAIN O MD!! R- renal A- Arthritis S- erositis (inflammation of the serous tissues of the body, pericardium, pleura, inner lining of abdominal cavity) H- hematologic
P- Photosensitivity
A- ANA positive
I- Immunologic
N- Neurologic
O- Oral ulcers
M– Malar rash
D- discoid rash
4/11 needed
Neonbatal lupus is due to the transplacental transfer of
Ro (SSA) and La (SSB)
WHat is the antibody that everyone with Lupus has?
ANA (anti-nuclear antibody)
Lupus antibodies
ANA- everyone has it
dsDNA- renal disease in lupus
RoSSA- neonatal lupus
La SSB- neonatal lupus
Anti SM- (smith antibody) very sensitive for lupus
Anti phospholid (anticardiolipin)- clots, abortions
Clinical lupus check-up
Are you having any problems with allopecia, rashes arthritis?
Do you have any trouble taking a deep breath (pleurisy)
We’re gonna do a urnalysis (rbc cast, proteinuria) CBC (-penia, complements)
What is key in the lupus pathogenesis
DEFECTIVE CLEARING OF ANTIGEN-ANTIBODY COMPLEX
GOTTA KNOW THAT
We don’t know what really causes itCDomplement
WHere are the autoantibodies coming from in SLE
B cells….these suckers avoid apoptosis and produce bad abs
T cells….increased helpers and decreased suppresors
Complement activation is huge in Lupus, for example, in the kidney
Immune complexes can form in the circulation and then deposit in the kidney, they may also form in situ
What is the major cause of the hemolytic anemia, thrombocytopenia, and vasculitis in SLE
Antibody directed Cell mediated Cytotoxicity (ADCC)
Basically, antibody binds to cells, Fc receptors on NATURAL KILLER CELLS bind, target cell dies
Anytime you hear ADCC
think NK cells
What the fuck is anti-phospholipid syndrome
You get antibodies to the phospholipids involved in clotting. Leads to a jacked up PTT time but somehow a hypercoaguable state.
It is significant because it can lead to thrombosis and recurrent fetal loss
Sjogren’s syndrome is divided into what two types
primary and secondary
What are teh three big clinical hallmarks
kerratoconjunctivitis sicca
parotid gland swelling
xerostima
Extraglandular features of sjogrens
fatigue, Raynaud’s, polyarthritis, arthralgias, ILD, neuropathy, purpura
Classic histological finding in Sjogren’s disease is?
mononuclear cell infiltrates in the lacrimal and salivary glands
90% of sjogrens pts are what sex?
female
What is Schirmer’s test?
a test which uses filter paper to document tear flow
Sjogren’s disease leads to a 44 fold increase in
risk for non-hodgkins lymphoma
What type of immune cell is the predominant player in sjogren’s syndrome?
T cells (CD4)
Common lab findings in Sjogren’s syndrome?
ANA, high rheumatoid factor, Anti-RoSSA, Anti- LA/SSB
