Spot Diagnosis

Spot Diagnosis Rheumatology Weekend 20-21 Nov. 2010

Describe, Dx, Ab asso

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

Diagnosis

Dermatomyositis •  Pathogenesis: Immune complex to vessel •  Ischemia due to vasculitis •  Pathology: perivascular and perifascicular infiltration (CD4); perifascicular fiber atrophy; degeneration and regeneration of muscle

Diagnosis, DDx

Calcinosis Cutis •  Abnormal calcification of subcutaneous tissue •  DDx: tophi, xanthoma, Heberden and Bouchard’s node

Diagnosis

Lupus Panniculitis •  •  •  • 

Panniculitis in LE Mostly found at face and limb girdles Post inflammation cause atrophy Lupus profundus

What test

Anti dsDNA •  Crithidia leuciliae •  Crithidia test is gold standard of anti dsDNA test •  ELISA test: titer relate to disease activity in LN

Diagnosis

Morphea •  Localized scleroderma •  Morphea type: patchy and ivory-colored •  Linear type: band-liked

What

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

What

Periungual Infarction •  Small to medium vasculopathy or vasculitis •  Seen in SLE, DM, SS, MCTD

Diagnosis

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

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

Diagnosis

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

What

Cytoid Bodies •  Retinal vasculitis •  Cause exudate and hemorrhage of fundus •  Relate to active vasculitis

How does it relate to SLE

Sapporo •  Sapporo Snow Festival •  APS meeting Sapporo criteria 1999 •  Nowadays: Sydney 2006

What

GAVE •  Gastric Antral Vascular Ectasia •  Vasculopathy manifestation •  Anemia in SSC

What and asso. Ab

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

What

Tophi •  Granuloma like: central micro-crystal and surrounding macrophage •  Alcohol fixation

Diagnosis

Eosinophilic Fasciitis •  Groove sign: furrows along fascia and blood vessels •  DDx in scleroderma-liked: EF; scleredema; nephrogenic systemic fibrosis; post CMT, bleomycin; GVHD

Antibody associated

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

Antibody related

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

What

Erythema Ab Igne •  Differential diagnosis of livedo reticularis •  Long term exposure to heat •  Reticulated hyperpigmentation

Diagnosis

Scleroderma •  Vasculopathy: intema media thickening •  Fibrosis: loss of normal architecture of dermal ridge, abnormal collagen bundle, loss of skin appendages

Diagnosis

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

How does she relate to SLE

Ultraviolet •  Trigger of SLE flare: UVB 290-310 nm •  Exposure of anti Ro and apoptosis of keratinocyte

What test

Tinel Sign •  Carpal tunnel syndrome •  Associate: diabetes, hypothyroid, RA, SSc crystal induced arthropathy, pregnancy, amyloidosis, mechanic use

Diagnosis

Sjogren Syndrome •  Sialogram: cherry-blossom appearance (budding) of salivary duct

How dose it relate to SS

Rose Bengal •  Rose Bengal dye •  Keratoconjunctivitis sicca •  Schirmer’ test: less than 5 mm. within 5 min. •  Saxon test: weighing of chewed-gauze

Diagnosis

Parry-Romberg Syndrome •  Progressive hemifacial atrophy •  Skin and bone atrophy •  Seizure

En Coup de Sabre

•  Long streak resembling sabre wound

Diagnosis

Pannus •  Lymphoid follicle-like in synovial tissue •  Cytokine-driven: TNF alpha, IL-1, IL-2, IL-6, IL-17, IL-23

Granuloma

How relate to rheu. disease

Saturnine Gout •  Lead poisoning and gout •  Roman God Saturn festival: heavy wine drinking from lead-lined goblet

Diagnosis

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

DDx

Saddle Nose •  Rheum.: WG, relapsing polychondritis •  Hem.: NK/T cell lymphoma •  ID.: leprosy

Diagnosis

Scleromalacia •  Episcleritis and scleritis are most common •  Thinning of sclera: scleromalacia •  Protrusion of vitreous body: scleromalacia perforans

Diagnosis

Anterior Uveitis •  •  •  • 

Ciliary injection and hypopyon Extra-articular symptom of SpA AS: more unilateral ReA: more bilateral

How dose picture relate to rheu.

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

Name of mab(s) against this

Rituximab/Ocrelizumab

Mab against this

Tocilizumab

Biological agents against this

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