Systemic rheum/muscular dz Flashcards
Polymyalgia rheumatica
70 yo, woman
proximal joint pain/stiffness - hips, shoulder girdle, neck
malaise, wt loss, fever
no weakness of muscles
Assoc w/ giant cell arteritis
Labs: elevated ESR, normal CK
Tx: prednisone
Polymyositis
CD8+ injury to muscle of shoulder and pelvic girdle
-muscle bx
Lab: Elevated CK and aldolase
+ANA, +anti-Jo1
10% risk of malignancy
Dermatomyositis
Muscle inflammation
Skin: malar rash, heliotrope rash, gottron’s papules, shawl sign, V sign
mechanics hands
Anti-Jo1+
15% risk malignancy
Diagnostic criteria of SLE
Need 4/11
Malar rash
discoid rash - atrophic scarring
photosensitivity
painless oral ulcers
arthritis - nonerosive - two joints
Serositis - pleuritis, pericarditis
+ANA - nonspecific
Renal: proteinuria, cellular casts Neuro: seizures, psychosis Heme: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia Immune: -Antiphopholipid Ab (aka anticardiolipin Ab) = hypercoag -anti-dsDNA Ab - specific -anti-Smith Ab - specific -false positive VDRL
SLE presentation
young, middle age women, black
fatigue, wt loss, fever LAD Splenomegaly Libman Sacks endocarditis - sterile vegetations, both sides of valve Raynauds
Tx of SLE
steroids - glucocorticoids
NSAIDs
Hydroxychloroquine
Advanced w/ nephritis - cyclophosphamide
Lupus nephritis
Leading cause of death in lupus patients
wire loop lesions, deposition of immunocompleses
“Wire lupus”
Why does SLE increase risk of severe infections?
decreased C3 and C4 –> immunodeficiency
Drug induced lupus
anti-histone Ab
Drugs: "SHIPP" Sulfonamides Hydrazine Isoniazid Phenytoin Procainamide
Scleroderma - diffuse
Anti-DNA topoisomerase I Ab - Anti-Scl70
widespread
rapidly progress
viscera involved early
Scleroderma
progressive systemic sclerosis
Fibrosis and excess collagen deposit –> skin tight, shine, no wrinkles
-can’t extend fingers/grip
CREST syndrome
Anti-centromere Ab
Limited scerloderma
Calcinoshs - subepithelial Ca2+ deposits Raynaud's Esophageal dysmotility Sclerodactylyl - scarring of fingers Telangectasias
Limited skin involvement, less visceral involvement
slower progression
Sjogren syndrome triad
Dry eyes - xerophthalmia: dryness, conjunctivitis, sensation of sand in eyes - lacrimal involvement
Dry mouth - xerostomia: dysphagia, difficulty swallowing - parotid enlargement b/l, increased dental caries
Arthritis
Sjogrens incidence, assoc sx, risk factors
women 50-60s
Anti-Ro (anti SSA)
Anti-La (anti SSB)
Dx: lip bx
increased risk of B cell lymphoma
Assoc sx: reflux esophagitis, xerotrachea - dry throat, nasal dryness, chronic bronchitis, vaginal dryness, dyspareunia
Fibromyalgia
Excess muscle tenderness in 11 of 18 trigger points
Chronic generalized pain (increased sensitivity to pain), fatigue, sleep disturbances, HA, cognitive difficulty, mood disturbances
30% depression and/or anxiety
tx:
Pharm: pregabalin (neuropathic pain), milnacipran (SNRI)
-amitriptyiline, low dose analgesic (APAP, NSAIDs), fluoxetine
Non pharm: reassurance, exercise, stretching, sleep, relaxation techniques, stress reduction