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 Bouchards 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 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