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38 Cards in this Set

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Lecture 7
Scleroderma and Myositis
Classically, how is systemic sclerodema divided up?
Diffuse (widespread skin disease that progresses rapidly and has early viceral involvment) and Limited ( CREST and pulmonary HTN)
What are the types of localized scleroderma?
Morphea (hardened skin), Linear Sclerodema, Eosinophilic fasciitis.
What are the clinical features of scleroderma? (9)
Skin tightening. Raynaud's phenomenon, Digital Ischemia, Ulcerations, Calcinosis. Renovascular disease, Cardiac disease. GI disease. Lung disease.
Wat are the different lung disease associated with scleroderma? (2)
Interstitial fibrosis (mostly seen in diffuse disease) and Pulmonary HTN (more common in limited disease)
What is the pathogenesis of Pulm HTN in scleroderma? (3)
Vascular dysfuncton results from 1: Endothelin (causing vasoconstriction and vascular proliferation), 2: loss of vasodilitory action of Nitroc oxide, 3: Loss of vasodilitory action of prostacyclins
How is scleroderma Pulm HTN detected?
History, Physial exam Lab tests, CXR and CAT scan, PFT (check for DLCO), ECHO (don't estimate PA pressure from ECHO cos not always accurate. Rather, use swan-ganz)
What are the autoantibodies in scleroderma (4)
ANA (positive 80% ), Anti-SCL-70 (against DNA topoisomerase --> helps to predict interstitial lung disease), Anti RNA polymerase Abs (indicates risk o f cardiac and renal disease), Anticentromere Ab (in 20 - 30 % of pt with limited disease)
How do your treat Pulm HTN in scleroderma (3)
1: Endothelin receptor antagonists (Ambrisentan, Bosentan), 2: PDE-5 inhibitors like Sildenafil (increases NO), 3: Prostacylins (Epoprostenol, Treprostinil, Iloprost --> these drugs are all IV and so given as last resort or if patient present with really bad disease)
How does it work (d-penicllinamine) ?
interferes with collagen crosslinking
How does it work (Methotrexate and Mycophenolate Mofetil) ?
used to treat autoimmune disease (suppresses immune system)
How does it work (Colchicine) ?
Microtubule polymerization/depolymerization. \Helps with calcinosis
How does it work(Steroids) ?
Immune suppressor. Used to treat edematous phase, Myositis, Interstitial lung disease (be careful in this case, because it can cause renal disease)
How does it work (PPI/ prokinetics) ?
Helps with gut motility and prevents erosion)
How does it work (cytoxan)
standardcare for interstitial lung disease
What is the best way to treat renal crisis in scleroderma?
Ace inhibitors and tight BP control.
What are the clinical features of polymyositis? (4)
Symmetric proximal muscle weakness, Elevated muscle enzymes (CPK, aldolase, transaminases, LDH), Myopathic EMD abnormalities, Typical changes on muscle biopsy
What are the clinical features of dermatomyositis (5)
Polymyositis + typcial rash of dermatomyositis
How do the myscle biopsy of dermatomyositis and polymyositis differ?
Dermatomyositis shows inflammatory infiltrates in the blood vessels that course the muscle while polymyositis shows inflammatory inflitrates within the musclle fibers.
Which areas of the body are most susceptible to dermatomyositis?
Sun-exposed areas
How do you treat dermatomyositis/polymyositis ?
methotrexate/ mycophenolate mofetil
Lecture 8
Soft-tissue Rheumatism
Diagnosis of Fibromyalgia
Widespread oain for at least 3 months. Pain on the right and left sides of the body. Pain above and belwo the waist. Pain th einte axial skeleton. Pain on palpitation with a 4-kg force in 11 of 19 different sites.
Lab findings in Fibromyalgia
Everything is normal
Causes of diffuse myalgias
medications. Viral infections. Hypthryroidism. Sleep apnea. Polymyalgia rheumatica
What is the pathogenisis of fibromyalgia
Genetic predisposition --> Initiating stimulus --> Dysregulation of NT function--> Diminished central sensitization --> decreased dopamine levels in the brain --> lowered pain threshold
How do you treat fibromyalgia
Stretching excercises, SSRIs and SNRIs (Duloxetine and Milnacipran), Gabaergic drugs (Pregabalin), Behavior modification.
Clinical picture of Polymyalgia Rheumatica
> 65 yrs old. Pain and stiffness in the pectoral and pelvic girdle (proximal muscles). Low grade fever. Rare association with giant cell arteritis. Normal strength with no rash. Elevated ESR and CRP + ACI, Mildly abnormal LFTs. More women than men.
Treatment of PMR
low doses of steriods for a minimum of 6 months (but longer is recommended). Use bisphosphonates to prevent osteoporosis while on steroids
Hallmarks of repetitive stress syndromes
History of new or repetitive overuse of a muscle group. Local tenderness. Pain improves with gradual stretching. Usually improves with time. May have dystrophic calcification (this wont' get better on it's own). Respond to local injections of steroids adn physical measurs (splinting, heat, stretching etc)
Subacromial bursitis
House painters. Pain with abduction and external rotation
Lateral epicondylitis
Tennis elbow. Pain with stress on brachioradialis tendon. Can treat with changin the muscle insertion point
DeQuervain's syndrome
Tenosynovitis of the extensor hallucis longus tendon sheath. Finkelstein's sign is positive
Sclerosing tenosynovitis of the finger flexor tendons
Frequent in diabetics. Frequent in musicians
Anserine Bursitis
Confused with and accompanied by artitis of the knee. Associated with obesity
Achilles tendonitis
Runners and dancers. Associated with quinolone antibiotics
Plantar fasciitis
Runners. Improved with orthotics and stretching
Carpal tunnel syndrome
Median nerve entrapment. Tinel's sign.