Rheum 2 Flashcards
What is SLE
AI disorder affecting multiple organs that has relapses and remissions
MC in young women and black women
*Put this on your DDx for anyone w/ multisystem disease and +ANA
What is the pathophys of SLE
chronic inflammation to relatively every organ
Autoantibodies to nuclear antigens
Sx 2/2 trapping antigen-antibody complexes in capillaries OR antibody destruction or host cells (platelets)
RF for SLE are
15-40 y/o
Women
Black
Genetic predisposition (HLA-DR2/3, +ANA, other rheumatic diseases)
How does SLE present clinically
Relapsing remitting pattern **Fatigue! Fever, anorexia, weight loss, malaise, skin lesions *Arthralgias, myalgias, pleural effusion vascular manifestation (Raynaud's, VTE) Pericarditis Seizure, psychosis conjunctivitis, photophobia anemia, leukopenia, LAD Lupus nephritis (hematuria, proteinuria)
What skin lesions are associated with SLE
*Butterfly rash (malar distribution)* Photosensitive Small vessel vasculitis (purpura, petechiae, splinter hemorrhage, etc.) Discoid plaques Periungal erythema Nail fold infarcts Alopecia
How do you diagnose lupus
Presence of Sx (H&P) + Labs
-need 4 or more criteria
You need 4 of the following 11 criteria to diagnose SLE
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
Kidney disease (>.5g proteinuria, casts, or 3+ dipstick proteinuria)
Neurologic disease (Sz or psychosis)
Hematologic disease (hemolytic anemia, leukopenia <4, Lymphopenia <1500, thrombo <100)
Immunologic abnormality (Abs to native DNA/Sm/APA)
Positive ANA
What labs are used in diagnosing SLE
\+ANA Reflex to antibodies- **Anti-dsDNA**, Anti-Sm*, Anti-ro/la/U1 Diminished serum compliment Anemia, leukopenia, thrombocytopenia BUN, Cr, UA ESR, CRP
MC lab abnormalities in SLE are
Anemia, Hypocomplementemia, ANA, Anti-native DNA
How do you Tx SLE
Sun protection, exercise NSAIDs Antimalarials (hydroxychloroquin) Corticosteroids (topical for skin, PO for flares) Methotrexate (arthritis) Immunosuppressives Resistant: Belimumab
What should you monitor SLE
Atherosclerosis, pulmonary HTN, antiphospholipid syndrome, Osteoporosis/osteopenia
What provokes flares in SLE many times
Sulfa drugs!
A lot of people with SLE are allergic to them
Most M&M in SLE is 2/2
Glomerulonephritis
CNS disease
Antiphospholipid antibodies
Variants of SLE are
Acute Cutaneous LE
Subacute Cutaneous LE
Chronic Cutaneous LE: Discoid LE**
Drug induced lupus
Sx of ACLE are
Facial eruption
Generalized eruption
TEN
Sx of SCLE are
Small erythematous, scaly papules evolving into psoriasis plaques
Over shoulders, forearms, neck, upper torso
Photosensitivity
+ ANA
Arthralgias
Oral ulcers
Drug induced lupus
How does DLE present
Erythematous, indurated plaques covered with scale
Inflammation and scarring lesions
Over face, neck, scalp, ears
20% develop SLE
What drugs trigger Drug induced Lupus
Procaine Hydralazine Minocycline Diltiazem Penicillamine, Isoniazid, Quinidine, Methyldopa, Chlorpromazine, Practolol
How does Drug induced Lupus present
Fever malaise myalgias arthralgias ANA and Anti-histone + -Rare: cutaneous manifestation, renal or neuro involvement. Anti-DNA/SM are negative
How is pregnancy affected by SLE
High rate Spontaneous abortion, pre-term birth, IUGR
Passive autoimmunity )anti-SSA/SSB cross placenta
Neonatal lupus: Congenital heart block, cutaneous involvement that resolves in 6 mo.
What is antiphospholipid antibody syndrome
Autoimmune hypercoagulability disorder
Recurrent thromboses (venous or arterial)
Leads to pregnancy complications (miscarriage)
What are the types of APS
Primary: absence of other disease
Secondary: 2/2 other AI diseases (SLE)
How does APS usually present
Asx until:
Recurrent pregnancy loss, SVT, pulmonary embolism, CVA, Budd-chiari syndrome, Cerebral vein thrombosis, MI
What in “recurrent pregnancy loss” that may present in those with APS
Unexplained loss after first trimester
1+ premature births (<34) 2/2 Eclampsia or Pre-E
3+ unexplained fetal death in first trimester
How do you diagnose APS
1 clinical event + Positive Ab blood test
Positive APS antibody blood tests are
Anti-cardiolipin antibodies (false + syphilis test)
Antibody to beta-2-glycoprotein
Lupus anticoagulant antibody
How do you treat APS
Anticoagulation for life (Warfarin) to maintain INR 2-3
If pregnant: SubQ heparin and low dose aspirin
-Other: IV heparin, high dose corticosteroids, IVIG, plasmapheresis
What is Raynaud phenomenon
Syndrome of sudden onset digital ischemia
Initial phase: Excess vasoconstriction
Recovery phase: Vasodilation
What triggers raynaud’s phenomenon
Emotional stress, or cold temp!
Relieved by warmth
What are the types of Raynaud’s phenomenon
Primary (R disease): idiopathic, benign, symmetric, young women
Secondary (R syndrome): scleroderma, ulceration, gangrene, nail fold capillary changes
Primary MC than Secondary (D>S)
Causes of Raynaud’s (just the categories bc there are SO many damn causes)
Rheumatic diseases Neurovascular compression Occupational (vibration injury) Hematologic disorder Meda Endocrine disorder Miscellaneous
Clinical presentation of Raynaud’s is
Initial: well demarcated digital pallor or cyanosis (numb, stiff)
Recovery: intense hyperemia, rubor, throbbing, paresthesia, pain
Affects fingers**, toes, nose, ears
ASx between attacks!!!!
DDx for Raynaud’s is
Scleroderma (also Sx in secondary R)
SLE
Buerger disease
How do you treat Raynaud’s (non-pharm)
Wear gloves, mittens, or socks Keep body warm Moisturize skin Stop smoking Avoid sympathomimetic drugs (decongestants, diet pills, amphetamines)
How do you treat Raynaud’s (pharm)
1** CCB- Nifedipine, Amlodipine, Felodipine
PDE-5 inhibitor (sildenafil), topical nitroglycerin, Prazosin, Losartan, prostaglandin
Surgery: Sympathectomy when Sx severe and med therapy fails. Good for primary, sot secondary!
What is scleroderma (systemic sclerosis
Diffuse fibrosis of skin and internal organs 2/2 Unknown etiology
W>M, 20-40 y/o peak
What is the pathophys of Scleroderma
Deposition of collagen in skin, kidney, heart, lungs, stomach causing fibrosis of skin and organs
What are types of scleroderma
Diffuse: hands, face, trunk, proximal extremities develop skin changes- Kidney, interstitial lung, and cardiac Dz
Limited*MC: Face and hands, pulmonary HTN late in disease (CREST syndrome)
How does Scleroderma present
Skin changes
Polyarthralgias
Esophageal dysfunction
Hand and finger swelling, +/- spread to trunk and face
Localized scleroderma (morphea, linear rash)
What is CREST syndrome (limited scleroderma)
Calcinosis cutis Raynaud phenomenon Esophageal dysfunction Sclerodactyly Telangectasia
How do you diagnose Scleroderma
+ANA
+anti-centromere antibody in CREST
+anti-SCL70 in diffuse (worse outcome)
How do you treat scleroderms
No cure!
Monitor for HTN (indicates kidney involvement)
Organ specific Tx: PPI (reflux), ACE-I (renal), CCB (raynaud’s), Immunosuppressive (PHTN)
What is the prognosis of Scleroderma
Poor, 10 yea survival is 40-60
But, better if CREST syndrome (limited)
What is polymyositis and dermatomyositis
Inflammation of striated muscle, proximal muscle weakness, dermatomyositis
W>M, 40-50 (if >50 think paraneopalstic)
Lowest in Japanese, highest in African american
Etiology of P&D is
Unknown!
Possible 2/2 muscle inflammation, and associated with occult malignancy (ovary, lung, pancreas)
How does P&D present
Painless muscle weakness around neck, shoulders, hips
Trouble climbing stairs, or standing from seated
SOB
Cough
Dysphagia (choke while eating, aspiration)
Polyarthralgias
Muscle atrophy
What is one way to differentiate P&D form SCD
SCD: Choking as in difficulty getting bolus down 2/2 smooth muscle affected
P&D: Striated muscle so difficulty initial swallowing
Skin rash with dermatomyositis is
Malar
Heliotrope (peri-ocular erythema)
Gottron’s papules
Shawl sign (over sun exposed area)
How do you diagnose P&D
Elevated CK and aldolase (muscle enzymes) Electromyogram Muscle biopsy (definitve!)
Diagnostic criteria is
Proximal muscle weakness Elevated muscle enzymes Muscle biopsy evidence EMG changes Skin rash
How do you treat P&D
High dose steroids
Immunosuppressives/DMARDS (methotrexate, azathioprine)
What is Sjogren Syndrome
AI disorder associated with other connective tissue d/o
F>M, 40-60
Also called Sicca or Dry eye syndrome
What is the pathophys of Sjogrens
Autoantibodies destroy salivary and lacrimal glands= decreased saliva and tear production
How does Sjogrens present
Xerostomia
Dry eyes
Enlarged parotids
Systemic: dysphagia, pleuritis, small vessel vasculitis, obstructive airway Dz, neuropsych dysfunction, pancreatitis, RTA, chronic interstitial nephritis
How do you diagnose Sjogrens
+RF
+ANA
*+ anti-LA antibodies (antiSSB)
*+anti-RO antibodies (antiSSA)
*Lip biopsy (definitive, shows gland fibrosis)
+Shirmer test (eval tear secretion- pos if <5mm of filter paper is wet after 5 min)
Criteria for Sjogrens is
Ocular Sx (at least 1)- dry eyes >3 mo, FB sensation, artificial tears >3xday
Oral Sx (at least 1)-dry mouth >3mo, recurrent swollen salivary glands, need liquid to swallow dry food
Ocular sign (at least 1)- schirmer test, positive vital dye stain
Oral sign (at least 1)- Schirmer test, positive vital dye stain
Positive lip biopsy
Positive anti-SSA/SSB (Ro/La)
How do you treat Sjogrens
Sx Tx!
Avoid anticholinergics and antihistamines
Keep mucosa moist
Pilocarpine (increase salivary flow- muscarinic agonist, secretagogues)
Cyclosporine (restasis eye drops)
What is Rose Bengal stain
Vital dye stain for oral region
What is van Bijsterveld
Vital dye stain for Ocular
