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