Lupus Flashcards
What is lupus?
Chronic inflammatory autoimmune disease
What are the courses of lupus?
Benign with occasional flares Frequent relapses Remission Permanent organ damage Life-threatening complications
Patho of lupus?
- Abnormal immune response
- Pathogenic autoantibodies
- Deposit in tissue
What are the autoantibodies in lupus?
Antinuclear
Antiphospholipid
Anti-double-stranded DNA
What are part of the pathogenesis in lupus?
Genetics
Environmental triggers
Immunologic triggers
Hormonal
What is involved with gentics in lupus?
IRAK1 on X-chromosome
10% of SLE pts have family history
What is involved with enviromental factors in lupus?
Ultraviolet light
Drugs
- Procainamide
- Hydralazine
- Quinidine
- Isoniazid
What is involved with immunologic in lupus?
Viruses- Epstein Barr
Dietary factors
-amino acid: L-canavanine
What should you be concerned with in history within lupus?
Medications: procainamide, hydralazine Sunlight exposure Hair loss Raynaud’s phenomenon Antiphospholipid syndrome FH
What is involved with MSK in lupus?
Nonerosive in 2 or more peripheral joints Wrist MCP/PIP Pain/stiffness out of proportion to PE Some pt have Fibromyalgia
What is involved with kidneys in lupus?
Major cause of morbidity and mortality
Within 1st 2 yrs of onset
Leads to renal insufficiency and failure
Need renal biopsy for definitive diagnosis
What is involved with cardiopulmonary in lupus?
Pleuritis Parenchymal lung disease Pulmonary thrombosis Pericarditis Myocarditis Not part of diagnostic criteria
What is the major cause of death in SLE?
Accelerated artherosclerosis with vascular endothelial dysfunction
What are antiphospholipid antibodies involved with?
Pulmonary thrombosis
What are the constitutional signs in lupus?
fatigue malaise wt loss fever lymphadenopathy
What are the skin/hair signs in lupus?
malar rash discoid rash photosensitive rash Raynaud’s phenomenon alopecia
What are the msk signs in lupus?
polyarthritis
polyarthralgias
myositis
What are the kidney signs in lupus?
HTN
proteinuria
hematuria
renal failure
What are the CNS signs in lupus?
seizures psychosis stroke depression H/A
What are the CV signs in lupus?
pericarditis
myocarditis
conduction abnormalities
What are the GI signs in lupus?
hepatosplenomegaly pancreatitis
bowel vasculitis
What are the ocular signs in lupus?
retinal vasculitis
scleritis
What are the hematologic signs in lupus?
hemolytic anemia
thrombocytopenia
What is involved with neuropsychiatric in lupus?
Affects CNS, PNS and ANS
Seizures and psychosis are only sysptoms listed in ACR
Cognitive impairment = MOST COMMON manifestation
What is included in American College of Rheumatology: Classification Criteria?
- Malar rash
- Discoid rash
- Photosensitivity (60-100%)
- Oral ulcers
- Arthritis
- Serositis (pleuritic or pericarditis)
- Renal disorder
- Proteinuria (> 3+)
- Cellular casts (red cell; hemoglobin; granular; tubular or mixed) - Neurologic disorder
- Seizures or psychosis - Hematologic disorder
- Hemolytic anemia w/ reticulocytosis; leukopenia; lymphopenia; thrombocytopenia - Immunologic disorder
- ANA to double-stranded DNA; + antibody to Smith nuclear antigen; + Antiphospholipid antibody - Antinuclear antibody (ANA)
*****Need 4 or more of the 11 features
95% specific; 85% sensitive
What is your DDX with suspected lupus?
RA Mixed connective tissue disease Systemic vasculitis Neoplastic disorder Systemic infection
What are the labs used in lupus?
CBC with diff CMP ESR CRP PT PTT ANA UA
What imaging is used in diagnosis of lupus?
CXR - Pleural effusion - Pulmonary infiltrates ECG Echocardiogram
What is the initial approach to the treatment of lupus?
Screening
- Artherosclerotic risk factors- leading cause of m/m in SLE
Counseling
- Smoking cessation
Conservative vs medicinal treatment
- Will depend on the severity of disease
What is the conservative treatment in lupus?
Lifestyle modifications
- Avoid sunlight/ high SPF sunscreen
- Avoid tobacco
- Avoid fatigue
- Adequate sleep
What medications are used in treatment of lupus?
Ibuprofen
- Not in acute nephritis
Hydroxychloroquine
- Skin lesions
- Arthralgia
- Arthritis
- Alopecia
- Malaise
- ACR guidelines for pts with lupus nephritis
- Improved lipid profiles, decreases thrombosis and prevents flares
Topical glucocorticoids
- Isolated skin lesions
Systemic glucocorticoids
- Vary based on disease process
Immunosuppressive therapy (usually prescribed by Rheumatology)
- Cyclophosphamide
- Life or organ threatening manifestations
Methotrexate
What is the prognosis of lupus?
Newly dx
- 90% 5 yr survival rate
- Immunosuppressive therapy
85%
- 15 yr survival rate
Worse prognosis
- AA, Asian, and Hispanics
Leading cause of death in SLE patients
- Atherosclerosis
Table comparing antibodies.
Anti-dsDNA 60% 95%
specificity for SLE; fluctuates with dz; assc with glomerulonephritis
Anti-Smith 20-30%
99% specific for SLE
Anti-U1RNP 30%
mixed connective tissue dz
Anti-Ro/SSA 30%
Sjӧgren’s syndrome, photosensitivity
Anti-La/SSB 20%
Sjӧgren’s syndrome
Antihistone 70%
Drug induced lupus
Antiphospholipid 30%
Arterial and venous thrombosis; pregnancy morbidity
