醫五J組討論

指導老師：VS 謝松洲
報告學生：JC 陳錫安
 Patient information
• 陳O蓮 (F, 44y11m)
• 病例號：3147413
• 床號： T0-13C-17-01
• Underlying disease：
– Sjögren syndrome , diagnosed on 2013/01
– SLE
– HBV carrier
– Colon polyp status post scope excision for many
times
 Past history
• 2012/6
– Initial presentation：
Bilateral leg skin rash with dry eye
– The patient went to 國泰hospital
• 2013/1
– Dr.謝松洲's OPD
– The level of SSA & SSB were high
– Sjogren syndrome with Leukocytoclastic vasculitis
were impressed
– OPD f/u since then
 Lab data
CBC+PLT 2013/01/30 General
2013/01/30
BioChemistry
WBC(k/μL) 6.14
Alb(g/dL) 3.8
RBC(M/μL) 4.23 ALT(U/L) 18
HB(g/dL) 11.6 CRE(mg/dL) 0.5
HCT(%) 35.2 CRP(mg/dL) 0.14

MCV(fL) 83.2
DIC Profile 2013/01/30
MCH(pg) 27.4 D-Dimer
1.28
MCHC(g/dL) 33.0 (μg/mL FEU)

PLT(k/μL) 202
RDW-CV(%) 13.4
PS() -
 Lab data
2013/01/30

Cryoglobulin(*) Negative

IgG(mg/dL) 870.00

IgM
5.06
anticardiolipin(MPL)

Anti-SS-A(AU/ml) 263

Anti-SS-B(AU/ml) 61

ESR 1HR 12
 Past history
• 2013/7
– Tc-99m Sialoscintigraphy showed excretory
dysfunction at bilateral parotid glands
• 2013/9
– Progression of itchy skin rash following the URI
and tonsillitis
– Bilateral lower foot numbness
• 2013/10
– Oral steroid was added
 Past history
• 2013/12
– Came to ER due to headache
• 2014/8
– Colonoscopy on showed colitis
• In recent three month
– Bilateral lower leg skin rash progressed and
extend to bilateral arm and hip area
 Endoxan therapy
• 2014/9
– Elevated ESR, CRP, D-dimer
– Thrombocytopenia
• 2014/9/24
– Endoxan (I)
– Mini-pulse steroid
• 2014/10/23～2015/2/10
– Endoxan (II)～(VI) are infused in OPD
 Present illness
• 3/20
– Progressive skin rash, epigastralgia, leg edema
• 3/22～24 (Admission)
– Endoxan (VII)
• 3/24
– EGD due to epigastralgia
• 3/25
– Blanchable rash over palms
– Subsided on 3/27
 General 2014/ 2014/ 2014/ 2014/ 2014/ 2015/ 2015/ 2015/
BioChemistry 09/23 09/27 10/16 11/14 12/12 01/08 02/09 03/07
Alb(g/dL) 2.9 3.4 3.6 3.7 3.7 3.5 3.5 3.0

ALT(U/L) 23 23 29 30 23 74 63 41

LDH(U/L)

CRE(mg/dL) 0.5 0.5 0.6 0.8 0.8 0.8 0.8 0.9

CRP(mg/dL) 0.12 0.81 0.78 0.27 1.89 1.22 2.31

General
2015/03/27 2015/03/31 2015/04/09
BioChemistry
Alb(g/dL) 2.6 2.8 2.3

ALT(U/L) 26 24 29

LDH(U/L)

CRE(mg/dL) 0.6 0.8 1.3

CRP(mg/dL) 2.52 0.74

 2014/ 2014/ 2014/ 2014/ 2014/ 2015/ 2015/ 2015/
DIC Profile
09/23 09/27 10/16 11/14 12/12 01/08 02/09 03/07
D-Dimer
2.20 1.47 1.67 1.21 1.43 1.62 2.68 1.97
(μg/mL FEU)
2014/ 2014/ 2015/ 2015/ 2015/
ESR
11/14 12/12 01/08 02/09 03/07
ESR 1Hr
14 18 19 21 32
(mm /hr.)

2015/03/22
P-ANCA(IFA)(1:N) Negative
C-ANCA(IFA)(1:N) Negative
Anti-Nuclear Antibody(1:N) 1:40(-)
 Admission course
• 4/6
– Fever with dypnea
– CXR showed bilateral lower lung infiltration
20140920 Chest PA view 20140406 Chest PA view
 Admission course
• 4/7
– Suspect atypical pneumonia
– Ampicillin was shift to Ciprofloxacin
• 4/10
– F/u CXR showed bilateral diffused patch with
ground glass infiltration
– Rapid progression, suspect PJP, r/o TB
– CRP: 13.1
• 4/13
– Under nasal cannula, exertional dyspnea
20140410 Chest PA view 20140413 Chest PA view
 Lab data & Infection survey
2015/03/22 2015/04/06 2015/04/10 2015/04/12
C-Reactive
Protein 1.28 5.94 13.10 4.13
(mg/dL)

2015/04/12

Mycoplasma Pneumoniae IgM(*) Negative

2015/04/07

Influenza A+B Rapid Screening Test(*) Negative

Discussion
 D/D of skin rash
• Idiopathic
• Physical cause, insect bite
• Radiation
• Allergy
– Drug
• Infection
• Autoimmune disease
• Hereditary disorders
• Deficiency disorders
– Niacin deficiency http://en.diagnosispro.com/differential_diagnosis-
for/erythematous-generalized-rash/41825-154.html
 D/D of skin rash
• Non-blanchable skin rash
Extravasation of RBC
– Senile purpura
– Necrotizing vasculitis
– Small vessel vasculitis
• Rheumatoid vasculitis, septic vasculitis
– Scurvy: perifollicular
– Thrombocytopenia purpura.
– Idiopathic systemic capillary leak syndrome: recurring
attacks, ?hypovolemic shock, hemoconcentration, and
hypoalbuminemia.
– Mediterranean spotted fever
• Rickettsia conorii
 Anti-SSA & Anti-SSB
• Among 181 ANA positive patients in whom
clinical information was available
– Anti-SSA and/or anti-SSB antibodies were identified
• Disease associations
– Systemic lupus erythematosus (SLE) (45.3%)
– Primary Sjögren's syndrome (pSS) (14.4%)
– Scleroderma (8.8%)
– RA (7.7%), cutaneous lupus (7.7%)
– Dermatomyositis (2.2%).
Ann Rheum Dis. 2002 Dec;61(12):1090-4.
• Scleroderma and dermatomyositis
– Enriched among mono-Ro52 reactive serum samples
(34.2% and 10.5% respectively)
• Single reactivity towards Ro60 or anti-Ro60 with
anti-Ro52 predisposed for SLE
– (80.0% and 52.2% respectively)
• Triple reactivity towards Ro52, Ro60, and SSB was
primarily linked with SLE (55.8%) followed by pSS
(20.9%).
• Anti-SSA on immunodiffusion increased the
chance for SLE (62.8%)
• Isolated anti-SSB reactivity on immunodiffusion
was less indicative for SLE (14.3%) and
predisposed more for cutaneous lupus (23.8%)
and pSS (33.3%).
Ann Rheum Dis. 2002 Dec;61(12):1090-4.
 Sjögren syndrome
• A systemic chronic inflammatory disorder
characterized by lymphocytic infiltrates in
exocrine organs
• Most cases present with sicca symptoms
– Xerophthalmia (dry eyes)
– Xerostomia (dry mouth)
– Parotid gland enlargement

http://emedicine.medscape.com/article/332125-overview#showall