Lupus Flashcards
All hypersensitivity reactions are immune-mediated tissue injury resulting in a variety of diseases:
3 examples?
- Allergies
- Autoimmune disease
- A variety of other inflammatory disease
Types of hypersensivty reactions (Gell and Coombs) Describe what is included in the following reactions: 1. Type I? 1 2. Type II? 5 3. Type III? 3 4. Type IV? 2
- Immediate (anaphalactic; atopic)
2.
- Cytotoxic-Target cell receptors;
- platelet-thrombocytopenia
- Target fixed tissue antigen;
- Goodpastures,
- M.G.
- Immune complex;
- Vasculitis;
- SLE.
- Cell mediated
a) Tuberculin reaction
b) Cytotoxic cells destroy target cells-allograft rej.
- What is SLE?
- Deposition of what? And where?
- Can affect what parts of the body? 10
Is sometimes referred to as the “great imitator” because it affects many different body systems.
- Multisystem autoimmune disorder of unknown cause and is strongly associated with various autoantibodies.
- Deposition of antigen-antibody complexes along vascular and tissue basement membranes.
- Skin,
- joints,
- serosal surfaces,
- muscles,
- kidneys,
- heart,
- lung,
- CNS,
- red blood cells and
- platelets.
Is sometimes referred to as the “great imitator” because it affects many different body systems.
Influenced by combination of the following:
1. Genetic? 3
- Environmental? 4
1. Genetic: Chromosome 6: -HLA-DR3 and linked specificities -DR2 and -DQw1 increase risk by a factor of three!
- Environmental:
- UV light (rash)
- Bacterial and viral infections
- Drugs
- Abnormal stimulation of immune system
Factors that affect Lupus:
1. Sex hormones appear to play some role
Most cases of SLE develop after what and before what?
- Drug induced:
Lupus like syndrome
Which drugs? 2
- menarche, menopause
- Procainamide
- Hydralazine
Pathogenesis of SLE
3 components?
- B-lymphocyte hyperactivity with exaggerated antibody production.
- Antigen-antibody complexes deposited along basement membranes of vasculature and tissue.
- Localized inflammatory responses:
PP of SLE: Localized inflammatory responses include? 3
Course is characterized by what?
- complement
- neutrophil migration
- cell-mediated tissue injury.
exacerbations and remissions.
- As in other autoimmune disorders-
the immune system attacks what? - Result in what? 2
- Type III Autoimmune reaction caused by what?
- body cells
- tissues.
- inflammation and
- tissue damage.
- antibody-immune complex formation.
Most common symptoms of SLE:
1. Systemic? 2
- MOuth and nose? 1
- Muscles? 1
- Joints? 1
- Psychological? 2
- Face? 1
- Pleura? 1
- Pericardium? 1
- Fingers and toes? 1
- Low grade fever
- Photosensitivity
- Ulcers
- Muscles
- Arthritis
- Fatigue
- Loss of appetite
- Butterfly rash
- Inflammation
- Inflammation
- poor circulation
Clinical Manifestations
1. Constitutional? 5
- Skin? 1
- Systemic? 3
- Digits, Hair and nail? 6
- Constitutional
- Fatigue
- Fever
- Weight loss
- Malaise
- Anorexia - Skin
- “butterfly” rash (malar rash) - Systemic
- polyarthritis,
- arthralgias,
- joint pain - Alopecia
- Fingertip lesions
- Periungual erythema
- Nail fold infarcts
- Splinter hemmorhages
- Raynaud’s phenomenon (about 20% of patients)
Polyarthritis is symmetrical, but does not involve articular destruction.
Renal features of SLE
2 causes?
- Lupus Nephritis
2. Hypertension
Lupus nephritis can cause?
2
- Glomerulonephitis
2. Nephrotic syndrome
Nephrotic syndrome features? 2
- Proteinuria
2. Hyaline casts
What can be prognostic for renal manifestations of SLE?
Renal biopsy
SLE: Renal biopsy can be prognostic showing what?
4
What kind of renal failure is most common?
- glomerular sclerosis
- fibrous crescents
- interstitial fibrosis
- tubular atrophy = poor outcome.
Chronic renal failure more common than acute.
What will the cytology show on the renal biopsy for lupus nephritis?
“wire loop” nephritis
Ocular features of SLE
5
- Conjuntivitis
- Photophobia
- Transient or permanent monocular blindness
- Blurred vision
- Cotton wool spots on retina
Musculoskeletal features SLE
3
- Transient polyarthritis with symmetric involvement
small and large joints. - Osteonecrosis of hips.
- Fibromyalgia often present.
Musculoskeletal features of SLE: Transient polyarthritis with symmetric involvement
small and large joints.
- On examination of joints what will you not find?
- 10% develop what kind of deformities?
- What are not present?
- No signs of inflammation on examination of joints.
- 10% develop rheumatoid-like hand deformities.
- Bony erosions not present.
Pulmonary features SLE
Occurs in 40-50% of patients
6
- Transient basilar pneumonic infiltrates = “lupus pneumonitis”
- Pleural effusions
- Pleuritis
- Bronchopneumonia
- Restrictive lung disease
- Rare: alveolar hemorrhage with massive hemoptysis, death
Pulmonary features SLE: Transient basilar pneumonic infiltrates = “lupus pneumonitis” will present how? 3
- Nonproductive cough
- Dyspnea
- Hypoxemia
Cardiac features of SLE
Occurs in 30-40% of patients
4
- Pericarditis
- Myocarditis
- Increased muscle enzymes
- Libman-Sacks endocarditis
Cardiac features of SLE
1. Myocarditis features? 4
- Increased muscles enzymes: Which ones? 2
- Libman-Sacks endocarditis characterized by what?
- Tachycardia
- Ventricular arrhythmia
- Conduction problems
- CHF, Cardiomyopathy
- MB or CPK
- -V. surface vegetations on valves
Vegetation breakoff may allow colonization
Serositis of SLE
3 examples
What would diagnose this? 2
- Pleuritis
- Pericarditis
- Peritonitis
- Fluid: less than 3000 WBC/ mm3 = monocytes and lymphocytes
- Reduced complement
Vascular features of SLE
4
- Raynaud’s phenomenon
- Arterial or venous thrombosis
- Lupus vasculitis
- Livedo reticularis
What is lupus vasculitis?
Palpable purpuric lesions on the shins in a patient with lupus and necrotizing vasculitis of the skin, kidney, and brain.
What is Livedo reticularis?
Patient with lupus and antiphospholipid antibodies with livedo reticularis (manifested by a reddish-cyanic, reticular pattern of the skin) which has resulted in ulcer formation.
GI Tract Symptoms SLE
3
- Transient, nonspecific abdominal pain.
- Increased incidence of primary biliary cirrhosis with SLE.
- Vasculitis of mesentery can cause infarction or perforation of bowel with high mortality.
CNS Features SLE
1. Whats the most common neurophysical symptom?
- Other symtpoms? 8
- Why at higher risk for stroke?
- Severe headaches, most common neuropsychological symptom, sometimes with scotomata like migraines.
- Confusion,
- memory deficits,
- disorientation,
- hypomania,
- delirium
- schizophrenia.
- Grand mal seizures,
- temporal lobe seizures.
- Stroke: anti-phospholipid antibodies.
- Specific SLE-related deaths, such as those due to nephritis, usually occur within what time period?
- What are common etiologies of late mortality?
- What is now the most common cause of death in early active SLE?
- the first 5 years of symptom onset.
- -Cardiovascular disease and
-malignancy,
which may be related to chronic inflammation and cytoxic therapies, are common etiologies of late mortality. - Infectious complications related to active SLE and immunosuppressive treatment are now
SLE Laboratory
4
- CBC
- ESR and CRP
- UA
- Antibody testing
What will the CBC show for SLE?
4
- Anemia
- Leukopenia
- Thrombocytopenia
- Prolonged PTT
CBC in SLE
- Anemia: Which kinds? 3
- Leukopenia: which kind and expressed what?
- Thrombocytopenia: What levels?
- Prolonged PTT: from what?
- This could be a false positive test for what?
CBC
- Anemia
- Normocytic
- Normochromic
- Hemolytic - Leukopenia
- Lymphocytopenia common,
- indicative of disease activity - Thrombocytopenia
- 50-100 K - Prolonged PTT
- from antiphospholipid antibodies - false positive tests for syphilis
ESR and CRP in SLE will be?
UA might show? 3
- ESR and CRP
- Both elevated - UA
- Proteinuria
- RBC’s/WBC’s
- Cellular or hyaline casts
What antibody tests would we do for lupus?
8
- ANA – (especially rim, cartwheel or peripheral) sensitivity 99%, spec 49%
- Anti dsDNA (anti-deoxyribonuclic acid) **[double stranded]
- Anti ssDNA (Anti-deoxyribonuclic acid)
- Anti ENA [anti-extractable nuclear antigen]
- Anti SM **
- Anti RNP - Elevated nonspecific IgG, IgM levels
- Reduced complement levels (C3, C4)
- Decreased IgA levels
- Anti-histone - Drug-induced lupus (DIL) ANA antibodies often this type
ANA shapes?
4
- Rim or cartwheel
- Diffuse
- Speckled
- Nucleolar
Differential Diagnosis SLE
13
- Antiphospholipid Syndrome
- Fibromyalgia
- Hepatitis C
- Lyme disease
- Lymphoma, B-cell
- Mixed Connective-Tissue
- Disease
- Polyarteritis Nodosa
- Rheumatic Fever
- Rheumatoid Arthritis
- Scleroderma
- Serum sickness
- TTP
CRITERIA FOR DIAGNOSING SYSTEM LUPUS ERYTHEMATOSUS
11
- Characteristic rash across the cheek
- Discoid lesion rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Inflammation of membranes in the lungs, the heart, or the abdomen
- Evidence of kidney disease
- Evidence of severe neurologic disease
- Blood disorders, including low red and white blood cell and platelet counts
- Immunologic abnormalities
- Positive antinuclear antibody (ANA)
Note: A patient must experience four of the criteria before a doctor can classify the condition as SLE. These criteria, proposed by the American College of Rheumatology, are not to be relied upon solely for diagnosis, however.
Physical findings: SLE
4
- Butterfly (malar rash)
- Hypertension
- Alopecia
- Discoid lesions
Diagnosis SLE
Labs?
5
- ANA (particularly cartwheel, rim or peripheral)
- dsDNA
- anti Sm ribonuclear protein
- Hypocomplementemia.
- UA
What are we looking for with the UA with SLE? 4
- WBC
- RBC
- Proteinura
- Hyaline casts
Treatment for SLE is aimed at?
3
Treatment aimed at:
- Reducing inflammation
- Suppressing the immune system
- Closely following patients clinically to identify disease features as early as possible.
Patient education and prevention of disease flares:
5
- Sunscreen and protective clothing
- Protective, warm clothing
- Avoidance of vasoconstrictive drugs
- Psychological support
- Routine immunization
Pharm treatment for SLE?
6
- Glucocorticoids
- Antimalarials
- Immunosuppresants
- Iv Immunoglobulin
- Plasmapheresis (lupus nephritis)
- Belimumab (FDA approved 2011) fully human
monoclonal antibody. (benlysta)
Drug Induced Lupus Syndromes
- Primarily symptoms where? 3
- What makes this different from? 4
- Treatment?
- Primarily
- musculoskeletal,
- pulmonary, and
- polyserositic symptoms. - Renal and neurologic disease uncommon.
- Antibodies themselves not harmful.
- No hypocomplementemia.
- Anti-nuclear antibodies present, but return to normal with withdrawal of drug.
- Reversal with discontinuation of drug
Drug Induced Lupus Syndromes
Name the drugs in the following classes that could cause this?
1. Antihypertensives? 2
- Antiarrhythmics? 2
- Anticonvulsants? 4
Others?
- Hydralazine *
- Methyldopa *
- Procainamide *
- Quinidine *
- Dilantin
- Zarontin
- Primidone
- Tegratol
Miscellaneous: INH * Sulfanamides PCN Thorazine PTU Tagamet Levadopa B-Blockers
- What is discoid lupus?
- Involves plaque-like lesions of what areas? 3
- First appears as what?
- Later?
- ___% of discoid lupus patients have disease that involves the skin.
- Chronic cutaneous lupus
- head, scalp and neck.
- First appear as red, swollen patches or skin
- Later
- scarring,
- depigmentation,
- plugging of hair follicles. - 90
Discoid therapies?
3
Therapy with
- sunscreens,
- topical corticosteroids, and
- antimalarial agents is usually effective.
