Rheumatology II Flashcards
What is Systemic Lupus Erythematous?
Who is it most common in?
Chronic inflammation affecting almost every organ
Autoantibodies to nuclear antigens
Ages 15-40
Women
Black women
What is the most common symptoms you’ll see with SLE?
What skin lesion is most commonly associated with SLE
FATIGUE
Butterfly rash
Due to Photosensitivity
Diagnostic Criteria for SLE?
Requires 4 or more criteria
Malor rash Discoid rash Photosensitivity Oral ulcer Arthritis Serosistis (pleuritis/pericardisits) Kidney disease (hematuria, proteinuria) Neurologic disease (seizures, psychosis) Hematologic disorders Immunoligic abnormalities Positive ANA
Which lab is most indicative of SLE?
+ANA
Also more specifically
If you have a positive ANA what reflex test must be done?
Which finding will have the highest sensitivity and specificity
Anti-dsDNA** (high specificity and sensitivity)
Anti-Sm (high specificity)
Treatments for SLE
Sun protection, exercise NSAIDS Antimalarials Hydroxycholorquine, chloroquine – helpful for MSK and skin manifestations [remember eye exams] Corticosteroids Methotrexate for arthritis
What 3 things can characterise Acute Cutaneous Lupus Erythematous (ACLE)
Facial eruption, generalized eruption, or TEN
What things characterize Subacute Cutaneous Lupus?
scaly papules that evolve into psoriasiform plaques
Usually shoulders, forearms, neck, upper torso
Photosensitivity
Positive antinuclear antibodies
Arthralgias
Oral ulcers
Clinical Presentation of Discoid Lupus
Erythematous, mildly indurated plaques, covered with a scale
Inflammation and scarring lesions
Face, neck, scalp, ears
What complications can occur with SLE and pregnancy?
Higher rates of spontaneous abortion
Risk of preterm birth, IUGR
Acquired autoimmunity
What complications can be seen in Neonatal Lupus
Congenital heart block
Cutaneous involvement
What is Antiphospholipid Antibody Syndrome
Autoimmune, multisystem disrder
Hypercoagulability syndrome
Recurrent thromboses in venous or arterial circulation
What is the difference between Primary and Secondary Antiphospholipid Syndrome?
Primary
In the absence of other disease
Secondary
Occurs with other autoimmune disease
SLE
Clinical Presentation of Antiphospholipid Antibody Syndrome?
Asymptomatic until they experience:
Recurrent pregnancy loss
Unexplained fetal death after first trimester
1 or more premature births before 34 weeks due to eclampsia or preeclampsia
How is Antiphospholipid Antibody Syndrome diagnosed?
One clinical event + positive antibody blood test(s)
Anti-cardiolipin antibodies
Can produce false-positive test for syphilis
Antibodies to beta-2-glycoprotein
Lupus anticoagulant antibody
Antiphospholipid Antibody Syndrome treatment?
Anticoagulation for life - warfarin to maintain an INR of 2-3
What is Raynaud Phenomenon?
How is it caused?
Syndrome of paroxysmal digital ischemia
Initial phase: excessive vasoconstriction
Recovery phase: vasodilation
Clinical presentation of Raynaud Phenomenon?
Initial phase: demarcated digital pallor or cyanosis
Numbness, stiffness, pain decreased sensation
Recovery phase: intense hyperemia and rubor
Intense throbbing, paresthesia, pain
Primarily affects fingers
Resolves with warm temps or putting extremity in warm water
1st Line Treatment for Raynaud Phenomenon?
Calcium channel blockers - first line
Nifedipine, amlodipine, felodipine
What is Scleroderma?
What is the pathophysiology behind this?
Diffuse fibrosis of skin and internal organs
Deposition of collagen in skin, kidney, heart, lungs, stomach
Results in fibrosis of skin and organs
Clinical Presentation of Scleroderma: CREST
Calcinosis cutis Raynaud phenomenon Esophageal dysfunction Sclerodactyly Telangiectasias
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Labs for Scleroderma?
+ ANA in 90% of patients with diffuse scleroderma
+ anti centromere antibody in CREST syndrome (more specific)
Treatment for Scleroderma?
No cure
Monitor for HTN, as it signals kidney involvement
What is Polymyositis & Dermatomyositis (P&D)
Inflammation of striated muscle
Proximal skeletal muscle weakness
Dermatomyositis – skin manifestations
Clincal presentation of polymyositis and Dermatomyositis?
Insidious, painless muscle weakness around neck, shoulders, hips
SOB
Dysphagia
Choking while eating, aspiration
What 4 skin rashes are associated with Polymyositis and Dermatomyositis
Malar
Heliotrope
Gottron’s papules
Shawl sign
What labs can help diagnose Polymyositis and Dermatomyositis?
What is the most definitive diagnostic test?
Labs:
Elevated muscle enzymes
Creatine kinase (CK)
Aldolase
Muscle biopsy**
What is the pathophysiology of Sjӧgren Syndrome
Auto antibodies destroy salivary and lacrimal glands
Decreased production of saliva and tears
What is a definitive diagnostic test used to idenfity Sjogren Syndrome
Lip biopsy to evaluate for gland fibrosis
Treatment for Sjogren Syndrome
Avoid medications with anticholinergic and antihistamine effects
Keep mucosa moist
Pilocarpine can increase salivary flow (muscarinic agonists, secretagogue)
Cyclosporine can improve eye symptoms
Clinical presentation of Sjogren syndrome?
Xerostomia (dry mouth)
Xerophthalmia (dry eyes)
Enlarged parotid glands
What labs are used to diagnose Sjogren syndrome
Which are going to be most specific
ANA (95%)
Anti-Ro (ant-SSA)
Anti-La (ant-SSB)
What is a positive Shirmer test when it comes to Sjogren?
Wetting less than 5mm of filter paper in 5 minutes is positive for Sjogren