spot diagnosis
TRANSCRIPT
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Spot Diagnosis Rheumatology Weekend
20-21 Nov. 2010
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Describe, Dx, Ab asso
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Mechanic Hand
• Hyperkeratosis and fissure of radial side of 2nd to 4th fingers and ulnar side of thumb
• Associate with antisynthetase syndrome • Antibody associated: Anti aminoacyl tRNA
synthetase • Anti histidyl tRNA synthetase or anti Jo-1
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Diagnosis
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Dermatomyositis
• Pathogenesis: Immune complex to vessel • Ischemia due to vasculitis • Pathology: perivascular and perifascicular
infiltration (CD4); perifascicular fiber atrophy; degeneration and regeneration of muscle
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Diagnosis, DDx
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Calcinosis Cutis
• Abnormal calcification of subcutaneous tissue
• DDx: tophi, xanthoma, Heberden and Bouchard’s node
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Diagnosis
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Lupus Panniculitis
• Panniculitis in LE • Mostly found at face and limb girdles • Post inflammation cause atrophy • Lupus profundus
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What test
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Anti dsDNA
• Crithidia leuciliae • Crithidia test is gold standard of anti
dsDNA test • ELISA test: titer relate to disease activity in
LN
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Diagnosis
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Morphea
• Localized scleroderma • Morphea type: patchy and ivory-colored • Linear type: band-liked
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What
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Secondary RP
• Clue of secondary RP Age of onset older than 30 Asymmetrical Abnormal nailfold capillary Abnormal serology Abnormal CBC or ESR/CRP Severe pain and/or develop digital ulcer, digital pitting, gangrene
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What
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Periungual Infarction
• Small to medium vasculopathy or vasculitis
• Seen in SLE, DM, SS, MCTD
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Diagnosis
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SSc-Pattern Nailfold Cap.
• Early: dilatation of capillary turn to giant capillary, well-preserved distribution
• Active: more dilatation and microhemorrhage, mild disorganization
• Late: almost absence dilatation and hemorrhage, ramified neovascularization and intense disorganization
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VEDOSS
• Very Early Diagnosis of SSc • Major criteria: SSc pattern nailfold cap.;
Serology; RP • Minor criteria: calcinosis cutis; esophageal
sphincter dysfunction; puffy fingers; digital ulcers; telangiectasia; ground-glass at chest HRCT
• Diagnosis: 3 majors or 2 majors and 1 minor
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Diagnosis
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Discoid LE
• Chronic cutaneous LE • Involve skin appendage cause atrophic
scar • Typical: erythema or hypertrophic patch,
papule, plaque with follicular plugging and some atrophic center and telangiectasia
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What
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Cytoid Bodies
• Retinal vasculitis • Cause exudate and hemorrhage of fundus • Relate to active vasculitis
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How does it relate to SLE
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Sapporo
• Sapporo Snow Festival • APS meeting Sapporo criteria 1999 • Nowadays: Sydney 2006
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What
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GAVE
• Gastric Antral Vascular Ectasia • Vasculopathy manifestation • Anemia in SSC
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What and asso. Ab
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Rheumatoid Nodule
• Granuloma like: central necrosis and palisading histiocytes
• Associate with RF and ACPA • Increase prevalence in A2756G
polymorphism of methionine synthase reductase gene
• MTX induced nodularis
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What
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Tophi
• Granuloma like: central micro-crystal and surrounding macrophage
• Alcohol fixation
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Diagnosis
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Eosinophilic Fasciitis
• Groove sign: furrows along fascia and blood vessels
• DDx in scleroderma-liked: EF; scleredema; nephrogenic systemic fibrosis; post CMT, bleomycin; GVHD
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Antibody associated
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Anti-Ro and Anti-La
• Neonatal lupus • Associate antibodies: anti Ro/SSA and
anti La/SSB • Manifestation: skin, hemato, liver, cardio • Fluorinated steroid: dexamethasone and
betamethasone for cardiac involvement
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Antibody related
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Antiphospholipid
• Anticardiolipin: 40 units or 99th percentile of IgG or IgM
• Anti β 2 glycoprotein I: 99th percentile of IgG or IgM
• Anti phosphatidylserine • Anti phosphatidylethanolamine
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What
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Erythema Ab Igne
• Differential diagnosis of livedo reticularis • Long term exposure to heat • Reticulated hyperpigmentation
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Diagnosis
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Scleroderma
• Vasculopathy: intema media thickening • Fibrosis: loss of normal architecture of
dermal ridge, abnormal collagen bundle, loss of skin appendages
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Diagnosis
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Sjogren Syndrome
• Inflammation of exocrine gland • Autoimmune process: alpha frodin • Anti Ro, anti La, RF • Sicca symptom of eyes and mouth • Mikculicz disease • CD4 infiltration
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How does she relate to SLE
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Ultraviolet
• Trigger of SLE flare: UVB 290-310 nm • Exposure of anti Ro and apoptosis of
keratinocyte
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What test
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Tinel Sign
• Carpal tunnel syndrome • Associate: diabetes, hypothyroid, RA, SSc
crystal induced arthropathy, pregnancy, amyloidosis, mechanic use
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Diagnosis
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Sjogren Syndrome
• Sialogram: cherry-blossom appearance (budding) of salivary duct
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How dose it relate to SS
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Rose Bengal
• Rose Bengal dye • Keratoconjunctivitis sicca • Schirmer’ test: less than 5 mm. within 5
min. • Saxon test: weighing of chewed-gauze
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Diagnosis
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Parry-Romberg Syndrome
• Progressive hemifacial atrophy • Skin and bone atrophy • Seizure
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En Coup de Sabre
• Long streak resembling sabre wound
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Diagnosis
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Pannus
• Lymphoid follicle-like in synovial tissue • Cytokine-driven: TNF alpha, IL-1, IL-2,
IL-6, IL-17, IL-23
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Granuloma
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How relate to rheu. disease
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Saturnine Gout
• Lead poisoning and gout • Roman God Saturn festival: heavy wine
drinking from lead-lined goblet
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Diagnosis
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Polyarteritis Nodosa
• PAN: medium-vessel vasculitis • HBV-related and non HBV-related • HBV-related treatment: antivirals, plasma
exchange and high-dose steroid • Non HBV-related treatment: high-dose
steroid and cyclophosphamide
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DDx
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Saddle Nose
• Rheum.: WG, relapsing polychondritis • Hem.: NK/T cell lymphoma • ID.: leprosy
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Diagnosis
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Scleromalacia
• Episcleritis and scleritis are most common • Thinning of sclera: scleromalacia • Protrusion of vitreous body: scleromalacia
perforans
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Diagnosis
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Anterior Uveitis
• Ciliary injection and hypopyon • Extra-articular symptom of SpA • AS: more unilateral • ReA: more bilateral
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How dose picture relate to rheu.
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Poliosis
• Poliosis: absence or decrease melanin of hair
• VKH: Vogt-Konayagi-Harada syndrome • Autoimmune against melanin-containing
organ • Neuro: meningitis, stroke-liked, CN palsy • Eye: uveitis • Skin: poliosis, vitiligo
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Name of mab(s) against this
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Rituximab/Ocrelizumab
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Mab against this
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Tocilizumab
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Biological agents against this
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Anti-TNF alpha
• Etanercept: fusion protein against soluble TNF receptor
• Infliximab: chimerical mouse-human mab • Adalimumab: fully-humanized mab • Golimumab: fully-humanized mab • Certolizumab : pegylated-humanized mab