2.4 Autoimmune Disorders Part 2 Flashcards
What are the components of the RASH OR PAIN mnemonic for SLE?
- Rash (malar)
- Arthritis
- Soft tissue/ serositis
- Heme DO
- Oral/ nasopharyngeal ulcers
- Renal dz
- Photosensitivity
- ANA
- Immunosuppressants
- Neurological DO
Who is the classic SLE patient?
Middle aged AA /hispanic female
Is the female:male ratio with SLE more or less pronounced at extremes of age?
Less
What is the general pathophysiology of SLE?
Antibodies directed against host nuclear material, leading to the activation of C3, C4, and CH50 complement
Which three components of complement are classically depleted with SLE?
CH50
C3
C4
What type of hypersensitivity rxn is SLE?
III
What is CH50?
Test that measures complement C1-C9 levels and ability for complement to lyse RBCs
What are the three classic antibodies that are seen in SLE?
ANA
Anti-smith (snRNP)
Anti-dsDNA
What is the classic antibody that is elevated with drug induced SLE?
Anti-histone
What is the pathophysiology behind ANA?
As cells are damaged, nuclear material is released, exposing B cells to nuclear material, and activating the adaptive immune system.
-Ag/ab complexes are then phagocytosed by APCs, and further activate the immune system
Patients with what deficiency have an increased risk of developing SLE?
C1q, C2, C4 deficiency
What are the components of C3 convertase?
C1, C4, C2
What is the major function of C3b?
Removal of ag/ab complex
What is the function of Cr1 on RBCs?
Binds C3b, and takes the bound ag/ab complex to the spleen for removal
A deficiency in what complement components predisposes patients to SLE?
C1q
C2
C4
When in particular are malar butterfly rashes present?
Upon exposure to sunlight
What are the characteristics of the discoid rash with SLE?
Circular rash with erythema and plaques
What are the different forms of serositis that can occur with SLE patients?
Pericarditis
Pleuritis
What are the neurological s/sx of SLE?
Psychosis or Seizures
What is the most severe form of kidney damage that can occur with SLE?
Diffuse proliferative glomerulonephritis
What are some of the hematological disorders that can occur with SLE? (3) What type of hypersensitivity reaction does this represent?
Anemia
Thrombocytopenia
Leukopenia
Type II
What is the specific heart abnormality that can occur with SLE? What is this?
Libman-Sacks endocarditis
-Wart-like vegetations on both side of a valve
What is the role of anti-dsDNA in the prognosis of SLE?
Follows with the course of disease, and is used to predict renal involvement
What, generally, are antiphospholipid antibodies?
Autoantibodies against proteins bound to phospholipids
What are the three major antiphospholipid antibodies?
- Anticardiolipin
- Lupus anticoagulant
- Anti-beta2-glycoprotein I
Which antibody commonly found in SLE will produce a false positive VDRL and RPR test? Why?
Anticardiolipin
-lyses of cells 2/2 treponema pallidum infx releases cardiolipin
Which antibody commonly found in SLE will produce a falsely elevated PTT?
Lupus anticoagulant
What is the antiphospholipid antibody syndrome associated with SLE?
- Antiphospholipid antibody + a hypercoagulable state
- Leads to DVTs, hepatic vein, placental and cerebral thromboses
What is the treatment for APA syndrome?
Lifelong anticoagulation
What happens to PTT with APA syndrome? What is odd about this?
Falsely elevated, despite being a hypercoagulable state
What are the top three causes of death in SLE (not in order)?
- CV dz
- Infx
- Renal dz
What are the three major drugs that cause SLE?
Hydralazine
Procainamide
Isoniazid
What is the anti-arrhythmic drug that classically causes SLE?
Procainamide
What is the anti-TB drug that classically causes SLE?
Isoniazid
What organs are rarely involved with drug induced SLE, as compared to normal SLE?
CNS
Renal
True or false: removal of the drug that causes SLE usually results in remission
True
What is the treatment for SLE?
- Avoid sunlight
- Glucocorticoids
- NSAIDs
- Hydroxychloroquine
What are the nephrotic and nephrotic syndromes most commonly encounters with SLE?
Nephritic = diffuse proliferative glomerulonephritis
Nephrotic = Membranous nephropathy
What, generally, is Sjogren’s syndrome?
Autoimmune destruction of lacrimal and salivary glands
What type of hypersensitivity reaction is Sjogren’s syndrome?
IV (lymphocyte mediated with fibrosis)
What is the classical presentation of Sjogren’s syndrome?
- Xerophthalmia
- Xerostomia
- Bilateral parotid enlargement
What are the antibodies that are found with Sjogren’s syndrome? What are these against?
SS-A (Ro)
SS-B (La)
Against ribonuclear proteins
Can’t chew a cracker, or dirt in eyes = ?
Sjogren’s
What is the most common autoimmune disease associated with Sjogren’s?
RA
True or false: rheumatoid factor can be elevated in Sjogren’s syndrome, regardless if the patient actually has RA or not
True
What is the major issue associated with Anti-SSA and SS-B?
Can cross the placenta and cause congenital complete heart block
What is the lymphocytic sialadenitis that is found with Sjogren’s syndrome?
Lymphocytes attacking the salivary glands of the mouth / lip / parotid
What are the three major criteria that are used to diagnose Sjogren’s syndrome?
- Xerophthalmia
- ANA, SSA/SS-B/ RF +
- Lymphocytic sialadenitis
What hematological malignancy are pts with Sjogren’s syndrome more susceptible to? How does this usually present?
- B cell lymphoma
- Enlargement of the parotid gland late in the disease course
Pt with Sjogren’s syndrome + unilateral parotid gland enlargement = ?
B cell lymphoma
Who is usually affected with systemic sclerosis?
Middle aged females
What, generally, is systemic sclerosis?
Sclerosis of the skin and visceral organs
What is the pathophysiology behind systemic sclerosis?
Fibroblast activation leads to deposition of collagen
What is the theory behind the development of systemic sclerosis?
Endothelial damage leads to an immune reaction against collagen, causing release of TGF-beta and PDGF, leading to sclerosis and continued inflammation
What are the two major cytokines that activate fibroblasts?
TGF-beta
PDGF
What are the components of limited sclerosis? (Formerly known as CREST syndrome)?
- Calcinosis
- Raynaud’s phenomenon
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasias
True or false: there is early visceral involvement with CREST syndrome?
False–late involvement
What is the antibody that is found in CREST syndrome?
Anti-centromere antibody
What are the associated symptoms from esophageal involvement of CREST?
GERD and decreased peristalsis
Is visceral involvement with diffuse systemic sclerosis early or late in onset?
Early
What are the GI sequelae of diffuse systemic sclerosis?
GERD
Esophageal dysmotility
Malabsorption
What are the pulmonary sequelae of diffuse systemic sclerosis?
Interstitial fibrosis and pHTN
What are the renal sequelae of diffuse systemic sclerosis? What is the treatment for this?
Scleroderma renal crises–acute onset of renal failure and HTN
ACEIs
What are the cardiac sequelae of diffuse systemic sclerosis?
HTN
Pericarditis
What is the antibody that if found with systemic sclerosis?
Anti-DNA topoisomerase I abs
What are the features of mixed CT disease?
Mixed features of SLE, systemic sclerosis, and polymyositis
What are the antibodies found in MCTD?
- ANA
- Anti-U1-RNP
