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實習醫學生|陳佳菁
指導老師|陳彥如醫師
指導老師|謝佳偉醫師
| 2019 . 01 . 31
VGHTC_IMRH
Case
Presentation
A 64-yr woman
suffered from
Persistent Dry Cough with 

Progressive DOE
for 3 months
陳 女 士 

病歷號隱匿

64 years old
1
2
3
4
5
Patient
Profile
Chief
Complaint
History of
Present Illness General Info
64-year-old Woman
Independent ADL
Occupation
Former Shopkeeper of Izakaya @Tokyo
Substance Use
No Alcohol, Tobacco, Betel Nut
Education
Senior High School
Socio-economy
Widowed, Good Economic Status
Second-Hand Smoking for 30 years
Admission Day - 2019/01/20
Persistent Dry Cough
with
Progressive DOE
for 3 months
Patient
Profile
Chief
Complaint
History of
Present Illness
How the
Serial Events
Initiated ?
1
Son, Daughter-in-Law, Grandchildren - Fever . Cough
Mycoplasma Pneumonia
* Only 1 Episode of Fever
AUG. 2018
CLUSTER EVENT
3
OCT. 2018
PROGRESSIVE DYSPNEA
2
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness
How the
Serial Events
Initiated ?
1
Son, Grandchildren - Fever . Cough
Mycoplasma Pneumonia
Family Well-Treatment and Recovery

*Fever up to 39’C for 4 hours
AUG. 2018
CLUSTER EVENT
32
Family Members were Disease-Free
While Prolonged Cough Persisted
NYHA Functional Class II
SEP. 2018
INTRACTABLE COUGH
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness
How the
Serial Events
Initiated ?
1
Son, Grandchildren - Fever . Cough
Mycoplasma Pneumonia
Family Well-Treatment and Recovery

*Fever up to 39’C for 4 hours
AUG. 2018
CLUSTER EVENT
3
Unable to Handle Daily Chores
Short of Breathiness even resting
X Posture, Diurnal Change
X Limb Swelling, Chest Tightness, Chest Pain, Palpitation, Wheezing…

NYHA Functional Class III - IV
OCT. 2018
PROGRESSIVE DYSPNEA
2
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness
How the
Serial Events
Initiated ?
1
Son, Grandchildren - Fever . Cough
Mycoplasma Pneumonia
Family Well-Treatment and Recovery

*Fever up to 39’C for 4 hours
AUG. 2018
CLUSTER EVENT
3
OCT. 2018
PROGRESSIVE DYSPNEA
2
Family Members were Disease-Free
While Purulent Sputum with
Prolonged Cough Persisted
SEP. 2018
INTRACTABLE COUGH
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness
Unable to Handle Daily Chores
Short of Breathiness even resting
X Posture, Diurnal Change
X Limb Swelling, Chest Tightness,
Chest Pain, Palpitation, Wheezing…

NYHA III - IV
Dyspnea on Exertion
掃地 吸地 拖地 擦地
煮飯澆花 洗碗 倒垃圾
爬樓梯 洗衣 曬衣 燙衣
那時的我,喘到都不能...
How the
Serial Events
Initiated ?
1
Son, Grandchildren - Fever . Cough
Mycoplasma Pneumonia
Family Well-Treatment and Recovery

*Fever up to 39’C for 4 hours
AUG. 2018
CLUSTER EVENT
3
OCT. 2018
PROGRESSIVE DYSPNEA
2
Family Members were Disease-Free
While Purulent Sputum with
Prolonged Cough Persisted
SEP. 2018
INTRACTABLE COUGH
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness
Unable to Handle Daily Chores
Short of Breathiness even resting
X Posture, Diurnal Change
X Limb Swelling, Chest Tightness,
Chest Pain, Palpitation, Wheezing…

NYHA III - IV
Chest HRCT
@20181016
Blood Test
@201810240.2mg/kg/day
台中慈濟胸腔科 中國附醫
2 MONTHS AFTER S/S
Ground

Glass

Infiltration
Consolidation

Air Bronchogram

Subpleural Sparing
Suspect
Organizing
Pneumonitis
Traction
Bronchiectasis
Interlobular Consolidation
Ground

Glass

Infiltration
Consolidation

Air Bronchogram

Subpleural Sparing
Suspect
Organizing
Pneumonitis
Traction
Bronchiectasis
Interlobular Consolidation
Ground

Glass

Infiltration
Consolidation

Air Bronchogram

Subpleural Sparing
Suspect
Organizing
Pneumonitis
Traction
Bronchiectasis
Interlobular Consolidation
Ground

Glass

Infiltration
Consolidation

Air Bronchogram

Subpleural Sparing
Suspect
Organizing
Pneumonitis
Traction
Bronchiectasis
Interlobular Consolidation
TRACTION BRONCHIECTASIS RETICULAR OPACITIES UPPER / MID LOBE INVOLVED
| HRCT CORONAL VIEW
VGHTC_IMRH
How the
Serial Events
Initiated ?
1
Son, Grandchildren - Fever . Cough
Mycoplasma Pneumonia
Family Well-Treatment and Recovery

*Fever up to 39’C for 4 hours
AUG. 2018
CLUSTER EVENT
3
OCT. 2018
PROGRESSIVE DYSPNEA
2
Family Members were Disease-Free
While Purulent Sputum with
Prolonged Cough Persisted
SEP. 2018
INTRACTABLE COUGH
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness
Unable to Handle Daily Chores
Short of Breathiness even resting
X Posture, Diurnal Change
X Limb Swelling, Chest Tightness,
Chest Pain, Palpitation, Wheezing…

NYHA III - IV
Chest HRCT
@20181016
Blood Test
@201810240.2mg/kg/day
台中慈濟胸腔科 中國附醫
2 MONTHS AFTER S/S
End of
Present
Illness Hx
6
Hospitalization on
2019/01/20
JAN. 2019
ADMISSION FOR SURVEY
4
Chinese Medicine Worked
Sjogren's Syndrome Suspected
NOV. 2018
S/S ALLEVIATED !!!
5
Visited VGHTC IMRH OPD for
Second Opinion regarding
Sjogren's Syndrome
DEC. 2018
NO DISCOMFORT
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness
Examination
@20181024
Cytoplasmic Stain ?
End of
Present
Illness Hx
6
Hospitalization on
2019/01/20
JAN. 2019
ADMISSION FOR SURVEY
4
Chinese Medicine Worked
Sjogren's Syndrome Suspected
NOV. 2018
S/S ALLEVIATED !!!
5
Visited VGHTC IMRH OPD for
Second Opinion regarding
Sjogren's Syndrome
DEC. 2018
NO DISCOMFORT
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness
Immunomodulation Agent
地方的中醫診所
End of
Present
Illness Hx
6
Hospitalization on
2019/01/20
JAN. 2019
ADMISSION FOR SURVEY
4
Chinese Medicine Worked
Sjogren's Syndrome Suspected
NOV. 2018
S/S ALLEVIATED !!!
5
Visited VGHTC IMRH OPD for
Second Opinion regarding
Sjogren's Syndrome
DEC. 2018
NO DISCOMFORT
VGHTC_IMRH
Patient
Profile
Chief
Complaint
History of
Present Illness中國附醫 台中榮總
Immunomodulation Agent
陳 女 士

病歷號隱匿

64 years old
1
2
Patient
Profile Past Medical Hx
Thyroid Goiter
Insomnia
w/ intermittent depressive mood
s/p partial thyroidectomy in 1999
currently in Euthyroidism
Lorazepam 2mg PO TID
Duloxetine Hydrochloride 30mg PO QD
陳 女 士

病歷號隱匿

64 years old
Patient
Profile Pedigree
Suicide, 57 y/o
Fall, 69 y/o
Accident, 49 y/o
Hyperthyroidism
Prostate CA, 63 y/o
AMI, 48 y/o
No Other Contributory Autoimmune Family History
陳 女 士

病歷號隱匿

64 years old
1
2
3
4
5
Patient
Profile Relevant Hx
Family Hx
Not contributory
Contact/Cluster Hx
Family Members : Son, Grandchild
- Mycoplasma Pneumonia
Drug Hx
URI Symtoms Relief Agent
Chinese Medication
Travel Hx
Not contributory
Vaccine/Allergy Hx
Not contributory
Thyroid Dz - Elder sister w/ Hyperthyroidism
Autoimmune - No other known Hx
No pets keeping
Easy, alert, oriented

No fever

No general malaise

No body weight loss
Review of
System GENERAL
1
| WARD
Review of System Physical Examination
HEENT
Postnasal Dripping

No headache

No rhinorrhea

No dysphagia
2
CV
No chest tightness

No chest pain

No limb swelling

No JVE
3
Mild DOE

Mild non-productive cough

Silent breath sound

No painful breathing

No night sweating
CHEST GU
No hesitancy

No burning or pain
urination

No nocturia

No urine amount change
GI
No heartburn

No N/V

No pain

No bowel habit
change
4 5 6
| WARD
Review of System Physical Examination
Review of
System
Weaker while doing chores

No myalgia or back pain

No numbness or tingling

No swelling or tender joint
MUSCULOSKELETAL HEMATOLOGY
No bruising tendency

No hemorrhagic spots

No unhealing wound

No frequent getting cold
SKIN
No rash, papule

No palpable mass

No hair or nail
change
7 8 9
| WARD
Review of System Physical Examination
Review of
System
| SICCA SYNDROME
Review of System Physical Examination
Review of
System
No daily, persistent, troublesome dry eyes for more than 3 months

No recurrent sensation of sand or gravel in the eyes

No use tear substitutes more than 3 times a dayOCULAR
ORAL
No daily feeling of dry mouth for more than 3 months

No frequently drink liquids to aid in swallowing dry food
| ACTIVITY OF DAILY LIFE
Review of System Physical Examination
Review of
System
BRUSHING UP/DOWN STAIR
LAUNDRY TOILET
BP - 109/71 mmHg

PR - 89 /min

RR - 18 /min

BT - 36.8’C

SpO2 : 95% R/A
VITAL SIGNS HEENT
No lymphadenopahy

Pink conjunctiva

Anicteric sclera

No sinus tenderness

No oral ulcer
GENERAL
Appears her age

Good hygiene

Cooperative attitude

No cardiopulmonary
distress
Physical
Examination
| WARD
Review of System Physical Examination
Bilateral coarse BS

No hyper-resonance

No egophony

Symmetric expansion

Smooth respiratory
pattern
CHEST SKIN
Mechanic's hand @
bilateral 2-3 fingers

No Gottron's sign

No Heliotrope
Eruption

No purpura or rashes
ABDOMEN
Flat, non-distended

Normactive bowel sounds

No bruits

Soft, no guarding or
tenderness

No abnormal dullness
| WARD
Review of System Physical Examination
Physical
Examination
| Sign of
Dermatomyositis
Bilateral 2-3 fingers

Radial side

Scaling . Pealing
Mechanic’s
HandM
L a b o r a t o r y R e s u l t s
- Increased interstitial
infiltration and haziness over
both lungs, more on bil.
lower lung field

- Blunting of right CP angle,
R/O pleural effusion or
pleural thickening
20190120
2081228
20181228
- Increased interstitial
infiltrations over bilateral
lower lung fields

- Bilateral CP angle blunting,
favor pleural effusion or
pleural change
20190120
Interstitial lung disease
Suspect Sjogren's syndrome
Tentative Diagnosis
| ADMISSION
VGHTC_IMRH
- s/p partial thyroidectomy in 1999

- without medication of eltroxin
Thyroid Goiter
- Euthyroidism status
- SSA +
- HRCT
Progressive Dyspnea on Exertion

HRCT - Interstitial Lung Disease
Problem Based Practice
喘
| BRIEFING
肺
VGHTC_IMRH
|Heart Failure with Acute Pulmonary Edema

|Acute Myocardial Infarction

|Cardiac Tamponade
Cardiovascular
|Sepsis

|Diabetic Ketoacidosis
Metabolic - Endocrine
|Diffuse parenchymal lung diseases

|Pneumonia

|Pneumothorax

|Pulmonary Embolism

|Acute Exacerbation of Asthma / COPD
Respiratory
|Anemia

|Neuropsychiatry
Hematology . Others
1 3
2 4
| DIFFERENTIAL DIAGNOSIS OF DYSPNEA
VGHTC_IMRH
|Heart Failure with Acute Pulmonary Edema

|Acute Myocardial Infarction

|Cardiac Tamponade
Cardiovascular
|Sepsis

|Diabetic Ketoacidosis
Metabolic - Endocrine
|Diffuse parenchymal lung diseases

|Pneumonia

|Pneumothorax

|Pulmonary Embolism

|Acute Exacerbation of Asthma / COPD
Respiratory
|Anemia

|Neuropsychiatry
Hematology . Others
1 3
2 4
| DIFFERENTIAL DIAGNOSIS OF DYSPNEA
VGHTC_IMRH
ILD
|RADIOLOGICALVERSUSHISTOLOGICALDIAGNOSISINUIPANDNSIP:SURVIVALIMPLICATIONS

FLAHERTY,THWAITE,KAZEROONI,ETAL;THORAX2003;58:143–148
Routine Labs
Recent & Old
CXR
Pulmonary

Function Tests
Ga-67 Scan
No Identified Potential Cause from Hx
Inhaled Inorganic Dust
Silicates

Carbon

Metals
Inhaled Organic Dust
Thermophillic fungi

Animal protein

Bacteria
Others
Gases . Fumes . Vapors
NonSpecific
Interstitial
Pneumonia
|SUSPECTORGANIZINGPNEUMONITIS
VGHTC_IMRH
NSIP
Pulmonary Function Test
20181106 20190123
FVC
1.24L (55%)

Moderately Severe
1.46L (56%)

Moderately Severe
DLCO
33%

Severe Decrease
45%

Moderate Decrease
No evidence of active
inflammation is
demonstrated in both
lung.



The mildly increased
gallium uptake in the
posterior regions of both
lungs may be due to mild
inflammation.
51
2019 / 01 / 24
Ga-67
Scan
VGHTC_IMRH
Blood Test
Latex test for

Cryptococcus Antigen
- Mycoplasma Pneumonia-IgM 310.0 UNITS/ML
Chlamydia pneumonia

IgM rapid test
- Aspergillus Galactomannan Ag 0.162
Legionella IF Ab < 1:128
Urine Culture
Legionella Antigen - Pneumococcal Antigen -
Sputum Culture
Acid Fast Stain
1+ @0122

- *3
PCR - Quantiferon Assay -
Sputum Culture Normal mixed flora presented
L a b o r a t o r y R e s u l t s
Immunologic Panel
Autoantibody
RF ANA dsDNA RO-52 SSB Ab RO-60
30.5 IU/mL
Positive, 1:160

Nucleoplasm - Fine
speckled
58.1 WHO units/mL
Positive

63 EliA U/ml
Negative

< 0.3 EliA U/ml
Positive

33 EliA U/ml
Myositis Specific Ab
Mi-2α - NXP2 - PM-Scl75 - PL-12 -
Mi-2β +/- SAE1 - Jo-1 - EJ +++
T1F1 - Ku - SRP +/- OJ -
MAD5 - PM-Scl100 - PL-7 - RO-52 +++
Thyroid Function
Free T4 10.7 pg/mL Anti-TG Ab < 20 IU/ML
TSH 4.86 uIU/mL Anti-TPO Ab 190 IU/ML
L a b o r a t o r y R e s u l t s
M M T
Right Left Right Left
Neck
Flexors 3 Quadriceps 4 4
Deltoid 4 4 - Ankle Dorsiflexors 5 5
Biceps 4 + 4 +
Gluteus
Medius 4 - 4
Wrist Extensors 5 5 Gluteus Maximus 4 + 4 +
M a n u a l M u s c l e Te s t i n g - 8
EMG
/
NCV
Motor conduction study showed normal responses over the right median
and ulnar nerves, including the F-wave latencies, median transcarpal and
ulnar across-elbow conduction study.

Sensory conduction study showed normal responses over the right
median, radial and ulnar nerves.

Needle EMG showed normal pattern over the right Deltoid muscle. 

Motor conduction study showed normal responses over the bil. peroneal
and tibial nerves, including the F-wave latencies.

Sensory conduction study showed normal response over the right sural
nerve.

H-reflex was symmetric in amplitude and latencies bilaterally.

Needle EMG showed normal pattern over the right Iliopsoas muscle.
| 20190124
CONCLUSIONS

NORMAL EMG/NCV STUDY

OVER THE UPPER AND LOWER LIMBS
肌
VGHTC_IMRH
No significant muscle edema
or enhancement of bil.
gluteal muscle and thigh
muscle can be detected.
Focal fat-stranding over the
subcutaneous region of the
left buttock, superficial to
left gluteal maximus muscle,
fasciitis can not be ruled out.
Right Lower Limb
MRI
EULAR / ACR classification criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies
7.0
Possible
Probable
Definite
Non-IIM
5.3-5.4
5.5-7.4
≥ 7.5
< 5.3
No Biopsy
EULAR / ACR classification criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies
7.0
Probable
Definite
5.5-7.4
≥ 7.5
No Biopsy
Possible
Non-IIM
5.3-5.4
< 5.3
眼 乾 口 乾 ?
No daily, persistent, troublesome dry eyes for more than 3 months

No recurrent sensation of sand or gravel in the eyes

No use tear substitutes more than 3 times a dayOCULAR
ORAL
No daily feeling of dry mouth for more than 3 months

No frequently drink liquids to aid in swallowing dry food
| 2019/01/22 | SS
Impression

Grade I-II decrease of
function of left parotid &
bilateral submandibular
glands
Sialoscintigraphy
S
2017 ACR–EULAR Classification Criteria for Primary Sjögren’s Syndrome
4≥ 4
Shirmer test :
2mm/5mins
| HOSPITAL COURSE
14.2 mg/kg/day
0.9 mg/kg/day
Admission Discharge
tRNA synthetase syndrome with predominant interstitial lung disease
Seconday Sjogren's syndrome
Final Diagnosis
| ADMISSION
VGHTC_IMRH
- s/p partial thyroidectomy in 1999

- without medication of eltroxin
Thyroid Goiter
- Euthyroidism status
- SSA +

- Schimer test +
- EJ +++, RO-52 +++

- HRCT
實習醫學生|陳佳菁
指導老師|陳彥如醫師
指導老師|謝佳偉醫師
| 2019 . 01 . 31
VGHTC_IMRH
謝謝您
Interstitial Pneumonia with
Autoimmune Features
| CLASSIFICATION CRITERIA
Morphologic
| HRCT - NSIP W/ OP OVERLAP
| UNEXPLAINED PFT
VGHTC_IMRH
Clinical
| DISTAL DIGITAL FISSURING
VGHTC_IMRH
Serologic
| RF ≥ 2X ULN
| ANTI-RO
| ANTI-TRNA SYNTHETASE (EJ)
VGHTC_IMRH
Laboratory Results
No Elevation of Myositis / Inflammatory Markers
Immunologic Panel
Autoantibody
RF ANA dsDNA RO-52 SSB Ab RO-60
30.5 IU/mL
Positive, 1:160

Nucleoplasm - Fine
speckled
58.1 WHO units/mL
Positive

63 EliA U/ml
Negative

< 0.3 EliA U/ml
Positive

33 EliA U/ml
Complement
C3 C4
116.5 mg/dl 23.2 mg/dL
Immunoglobin
IgG Total IgE
1611 mg/dl 80.4 kU/l
Inflammation
Index
Ferritin ESR
54.5 ng/ml 31 MM/HR
Others
Cellfree IL-2R
245.4 pg/ml
Laboratory Results
/ 20181228
Nailfold
Capillaroscopy
| 20190123

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20190131_IMRH case presentation_陳佳菁_V.4_Public

  • 2. A 64-yr woman suffered from Persistent Dry Cough with 
 Progressive DOE for 3 months
  • 3. 陳 女 士 
 病歷號隱匿
 64 years old 1 2 3 4 5 Patient Profile Chief Complaint History of Present Illness General Info 64-year-old Woman Independent ADL Occupation Former Shopkeeper of Izakaya @Tokyo Substance Use No Alcohol, Tobacco, Betel Nut Education Senior High School Socio-economy Widowed, Good Economic Status Second-Hand Smoking for 30 years Admission Day - 2019/01/20
  • 4. Persistent Dry Cough with Progressive DOE for 3 months Patient Profile Chief Complaint History of Present Illness
  • 5. How the Serial Events Initiated ? 1 Son, Daughter-in-Law, Grandchildren - Fever . Cough Mycoplasma Pneumonia * Only 1 Episode of Fever AUG. 2018 CLUSTER EVENT 3 OCT. 2018 PROGRESSIVE DYSPNEA 2 VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness
  • 6. How the Serial Events Initiated ? 1 Son, Grandchildren - Fever . Cough Mycoplasma Pneumonia Family Well-Treatment and Recovery
 *Fever up to 39’C for 4 hours AUG. 2018 CLUSTER EVENT 32 Family Members were Disease-Free While Prolonged Cough Persisted NYHA Functional Class II SEP. 2018 INTRACTABLE COUGH VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness
  • 7. How the Serial Events Initiated ? 1 Son, Grandchildren - Fever . Cough Mycoplasma Pneumonia Family Well-Treatment and Recovery
 *Fever up to 39’C for 4 hours AUG. 2018 CLUSTER EVENT 3 Unable to Handle Daily Chores Short of Breathiness even resting X Posture, Diurnal Change X Limb Swelling, Chest Tightness, Chest Pain, Palpitation, Wheezing…
 NYHA Functional Class III - IV OCT. 2018 PROGRESSIVE DYSPNEA 2 VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness
  • 8. How the Serial Events Initiated ? 1 Son, Grandchildren - Fever . Cough Mycoplasma Pneumonia Family Well-Treatment and Recovery
 *Fever up to 39’C for 4 hours AUG. 2018 CLUSTER EVENT 3 OCT. 2018 PROGRESSIVE DYSPNEA 2 Family Members were Disease-Free While Purulent Sputum with Prolonged Cough Persisted SEP. 2018 INTRACTABLE COUGH VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness Unable to Handle Daily Chores Short of Breathiness even resting X Posture, Diurnal Change X Limb Swelling, Chest Tightness, Chest Pain, Palpitation, Wheezing…
 NYHA III - IV Dyspnea on Exertion
  • 9. 掃地 吸地 拖地 擦地 煮飯澆花 洗碗 倒垃圾 爬樓梯 洗衣 曬衣 燙衣 那時的我,喘到都不能...
  • 10. How the Serial Events Initiated ? 1 Son, Grandchildren - Fever . Cough Mycoplasma Pneumonia Family Well-Treatment and Recovery
 *Fever up to 39’C for 4 hours AUG. 2018 CLUSTER EVENT 3 OCT. 2018 PROGRESSIVE DYSPNEA 2 Family Members were Disease-Free While Purulent Sputum with Prolonged Cough Persisted SEP. 2018 INTRACTABLE COUGH VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness Unable to Handle Daily Chores Short of Breathiness even resting X Posture, Diurnal Change X Limb Swelling, Chest Tightness, Chest Pain, Palpitation, Wheezing…
 NYHA III - IV Chest HRCT @20181016 Blood Test @201810240.2mg/kg/day 台中慈濟胸腔科 中國附醫 2 MONTHS AFTER S/S
  • 15. TRACTION BRONCHIECTASIS RETICULAR OPACITIES UPPER / MID LOBE INVOLVED | HRCT CORONAL VIEW VGHTC_IMRH
  • 16. How the Serial Events Initiated ? 1 Son, Grandchildren - Fever . Cough Mycoplasma Pneumonia Family Well-Treatment and Recovery
 *Fever up to 39’C for 4 hours AUG. 2018 CLUSTER EVENT 3 OCT. 2018 PROGRESSIVE DYSPNEA 2 Family Members were Disease-Free While Purulent Sputum with Prolonged Cough Persisted SEP. 2018 INTRACTABLE COUGH VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness Unable to Handle Daily Chores Short of Breathiness even resting X Posture, Diurnal Change X Limb Swelling, Chest Tightness, Chest Pain, Palpitation, Wheezing…
 NYHA III - IV Chest HRCT @20181016 Blood Test @201810240.2mg/kg/day 台中慈濟胸腔科 中國附醫 2 MONTHS AFTER S/S
  • 17. End of Present Illness Hx 6 Hospitalization on 2019/01/20 JAN. 2019 ADMISSION FOR SURVEY 4 Chinese Medicine Worked Sjogren's Syndrome Suspected NOV. 2018 S/S ALLEVIATED !!! 5 Visited VGHTC IMRH OPD for Second Opinion regarding Sjogren's Syndrome DEC. 2018 NO DISCOMFORT VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness Examination @20181024 Cytoplasmic Stain ?
  • 18. End of Present Illness Hx 6 Hospitalization on 2019/01/20 JAN. 2019 ADMISSION FOR SURVEY 4 Chinese Medicine Worked Sjogren's Syndrome Suspected NOV. 2018 S/S ALLEVIATED !!! 5 Visited VGHTC IMRH OPD for Second Opinion regarding Sjogren's Syndrome DEC. 2018 NO DISCOMFORT VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness Immunomodulation Agent 地方的中醫診所
  • 19. End of Present Illness Hx 6 Hospitalization on 2019/01/20 JAN. 2019 ADMISSION FOR SURVEY 4 Chinese Medicine Worked Sjogren's Syndrome Suspected NOV. 2018 S/S ALLEVIATED !!! 5 Visited VGHTC IMRH OPD for Second Opinion regarding Sjogren's Syndrome DEC. 2018 NO DISCOMFORT VGHTC_IMRH Patient Profile Chief Complaint History of Present Illness中國附醫 台中榮總 Immunomodulation Agent
  • 20. 陳 女 士
 病歷號隱匿
 64 years old 1 2 Patient Profile Past Medical Hx Thyroid Goiter Insomnia w/ intermittent depressive mood s/p partial thyroidectomy in 1999 currently in Euthyroidism Lorazepam 2mg PO TID Duloxetine Hydrochloride 30mg PO QD
  • 21. 陳 女 士
 病歷號隱匿
 64 years old Patient Profile Pedigree Suicide, 57 y/o Fall, 69 y/o Accident, 49 y/o Hyperthyroidism Prostate CA, 63 y/o AMI, 48 y/o No Other Contributory Autoimmune Family History
  • 22. 陳 女 士
 病歷號隱匿
 64 years old 1 2 3 4 5 Patient Profile Relevant Hx Family Hx Not contributory Contact/Cluster Hx Family Members : Son, Grandchild - Mycoplasma Pneumonia Drug Hx URI Symtoms Relief Agent Chinese Medication Travel Hx Not contributory Vaccine/Allergy Hx Not contributory Thyroid Dz - Elder sister w/ Hyperthyroidism Autoimmune - No other known Hx No pets keeping
  • 23. Easy, alert, oriented
 No fever
 No general malaise
 No body weight loss Review of System GENERAL 1 | WARD Review of System Physical Examination HEENT Postnasal Dripping
 No headache
 No rhinorrhea
 No dysphagia 2 CV No chest tightness
 No chest pain
 No limb swelling
 No JVE 3
  • 24. Mild DOE
 Mild non-productive cough
 Silent breath sound
 No painful breathing
 No night sweating CHEST GU No hesitancy
 No burning or pain urination
 No nocturia
 No urine amount change GI No heartburn
 No N/V
 No pain
 No bowel habit change 4 5 6 | WARD Review of System Physical Examination Review of System
  • 25. Weaker while doing chores
 No myalgia or back pain
 No numbness or tingling
 No swelling or tender joint MUSCULOSKELETAL HEMATOLOGY No bruising tendency
 No hemorrhagic spots
 No unhealing wound
 No frequent getting cold SKIN No rash, papule
 No palpable mass
 No hair or nail change 7 8 9 | WARD Review of System Physical Examination Review of System
  • 26. | SICCA SYNDROME Review of System Physical Examination Review of System No daily, persistent, troublesome dry eyes for more than 3 months
 No recurrent sensation of sand or gravel in the eyes
 No use tear substitutes more than 3 times a dayOCULAR ORAL No daily feeling of dry mouth for more than 3 months
 No frequently drink liquids to aid in swallowing dry food
  • 27. | ACTIVITY OF DAILY LIFE Review of System Physical Examination Review of System BRUSHING UP/DOWN STAIR LAUNDRY TOILET
  • 28. BP - 109/71 mmHg
 PR - 89 /min
 RR - 18 /min
 BT - 36.8’C
 SpO2 : 95% R/A VITAL SIGNS HEENT No lymphadenopahy
 Pink conjunctiva
 Anicteric sclera
 No sinus tenderness
 No oral ulcer GENERAL Appears her age
 Good hygiene
 Cooperative attitude
 No cardiopulmonary distress Physical Examination | WARD Review of System Physical Examination
  • 29. Bilateral coarse BS
 No hyper-resonance
 No egophony
 Symmetric expansion
 Smooth respiratory pattern CHEST SKIN Mechanic's hand @ bilateral 2-3 fingers
 No Gottron's sign
 No Heliotrope Eruption
 No purpura or rashes ABDOMEN Flat, non-distended
 Normactive bowel sounds
 No bruits
 Soft, no guarding or tenderness
 No abnormal dullness | WARD Review of System Physical Examination Physical Examination
  • 30. | Sign of Dermatomyositis Bilateral 2-3 fingers
 Radial side
 Scaling . Pealing Mechanic’s HandM
  • 31. L a b o r a t o r y R e s u l t s
  • 32. - Increased interstitial infiltration and haziness over both lungs, more on bil. lower lung field
 - Blunting of right CP angle, R/O pleural effusion or pleural thickening 20190120 2081228 20181228 - Increased interstitial infiltrations over bilateral lower lung fields
 - Bilateral CP angle blunting, favor pleural effusion or pleural change 20190120
  • 33. Interstitial lung disease Suspect Sjogren's syndrome Tentative Diagnosis | ADMISSION VGHTC_IMRH - s/p partial thyroidectomy in 1999
 - without medication of eltroxin Thyroid Goiter - Euthyroidism status - SSA + - HRCT
  • 34. Progressive Dyspnea on Exertion
 HRCT - Interstitial Lung Disease Problem Based Practice 喘 | BRIEFING 肺 VGHTC_IMRH
  • 35. |Heart Failure with Acute Pulmonary Edema
 |Acute Myocardial Infarction
 |Cardiac Tamponade Cardiovascular |Sepsis
 |Diabetic Ketoacidosis Metabolic - Endocrine |Diffuse parenchymal lung diseases
 |Pneumonia
 |Pneumothorax
 |Pulmonary Embolism
 |Acute Exacerbation of Asthma / COPD Respiratory |Anemia
 |Neuropsychiatry Hematology . Others 1 3 2 4 | DIFFERENTIAL DIAGNOSIS OF DYSPNEA VGHTC_IMRH
  • 36. |Heart Failure with Acute Pulmonary Edema
 |Acute Myocardial Infarction
 |Cardiac Tamponade Cardiovascular |Sepsis
 |Diabetic Ketoacidosis Metabolic - Endocrine |Diffuse parenchymal lung diseases
 |Pneumonia
 |Pneumothorax
 |Pulmonary Embolism
 |Acute Exacerbation of Asthma / COPD Respiratory |Anemia
 |Neuropsychiatry Hematology . Others 1 3 2 4 | DIFFERENTIAL DIAGNOSIS OF DYSPNEA VGHTC_IMRH
  • 37. ILD
  • 39.
  • 40.
  • 41. Routine Labs Recent & Old CXR Pulmonary
 Function Tests Ga-67 Scan
  • 42.
  • 43.
  • 44. No Identified Potential Cause from Hx Inhaled Inorganic Dust Silicates
 Carbon
 Metals Inhaled Organic Dust Thermophillic fungi
 Animal protein
 Bacteria Others Gases . Fumes . Vapors
  • 45.
  • 46.
  • 47.
  • 49.
  • 50. Pulmonary Function Test 20181106 20190123 FVC 1.24L (55%)
 Moderately Severe 1.46L (56%)
 Moderately Severe DLCO 33%
 Severe Decrease 45%
 Moderate Decrease
  • 51. No evidence of active inflammation is demonstrated in both lung.
 
 The mildly increased gallium uptake in the posterior regions of both lungs may be due to mild inflammation. 51 2019 / 01 / 24 Ga-67 Scan VGHTC_IMRH
  • 52. Blood Test Latex test for
 Cryptococcus Antigen - Mycoplasma Pneumonia-IgM 310.0 UNITS/ML Chlamydia pneumonia
 IgM rapid test - Aspergillus Galactomannan Ag 0.162 Legionella IF Ab < 1:128 Urine Culture Legionella Antigen - Pneumococcal Antigen - Sputum Culture Acid Fast Stain 1+ @0122
 - *3 PCR - Quantiferon Assay - Sputum Culture Normal mixed flora presented L a b o r a t o r y R e s u l t s
  • 53. Immunologic Panel Autoantibody RF ANA dsDNA RO-52 SSB Ab RO-60 30.5 IU/mL Positive, 1:160
 Nucleoplasm - Fine speckled 58.1 WHO units/mL Positive
 63 EliA U/ml Negative
 < 0.3 EliA U/ml Positive
 33 EliA U/ml Myositis Specific Ab Mi-2α - NXP2 - PM-Scl75 - PL-12 - Mi-2β +/- SAE1 - Jo-1 - EJ +++ T1F1 - Ku - SRP +/- OJ - MAD5 - PM-Scl100 - PL-7 - RO-52 +++ Thyroid Function Free T4 10.7 pg/mL Anti-TG Ab < 20 IU/ML TSH 4.86 uIU/mL Anti-TPO Ab 190 IU/ML L a b o r a t o r y R e s u l t s
  • 54. M M T Right Left Right Left Neck Flexors 3 Quadriceps 4 4 Deltoid 4 4 - Ankle Dorsiflexors 5 5 Biceps 4 + 4 + Gluteus Medius 4 - 4 Wrist Extensors 5 5 Gluteus Maximus 4 + 4 + M a n u a l M u s c l e Te s t i n g - 8
  • 55. EMG / NCV Motor conduction study showed normal responses over the right median and ulnar nerves, including the F-wave latencies, median transcarpal and ulnar across-elbow conduction study.
 Sensory conduction study showed normal responses over the right median, radial and ulnar nerves.
 Needle EMG showed normal pattern over the right Deltoid muscle. 
 Motor conduction study showed normal responses over the bil. peroneal and tibial nerves, including the F-wave latencies.
 Sensory conduction study showed normal response over the right sural nerve.
 H-reflex was symmetric in amplitude and latencies bilaterally.
 Needle EMG showed normal pattern over the right Iliopsoas muscle. | 20190124 CONCLUSIONS
 NORMAL EMG/NCV STUDY
 OVER THE UPPER AND LOWER LIMBS 肌 VGHTC_IMRH
  • 56. No significant muscle edema or enhancement of bil. gluteal muscle and thigh muscle can be detected. Focal fat-stranding over the subcutaneous region of the left buttock, superficial to left gluteal maximus muscle, fasciitis can not be ruled out. Right Lower Limb MRI
  • 57. EULAR / ACR classification criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies 7.0 Possible Probable Definite Non-IIM 5.3-5.4 5.5-7.4 ≥ 7.5 < 5.3 No Biopsy
  • 58. EULAR / ACR classification criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies 7.0 Probable Definite 5.5-7.4 ≥ 7.5 No Biopsy Possible Non-IIM 5.3-5.4 < 5.3
  • 59. 眼 乾 口 乾 ? No daily, persistent, troublesome dry eyes for more than 3 months
 No recurrent sensation of sand or gravel in the eyes
 No use tear substitutes more than 3 times a dayOCULAR ORAL No daily feeling of dry mouth for more than 3 months
 No frequently drink liquids to aid in swallowing dry food
  • 60. | 2019/01/22 | SS Impression
 Grade I-II decrease of function of left parotid & bilateral submandibular glands Sialoscintigraphy S
  • 61. 2017 ACR–EULAR Classification Criteria for Primary Sjögren’s Syndrome 4≥ 4 Shirmer test : 2mm/5mins
  • 62. | HOSPITAL COURSE 14.2 mg/kg/day 0.9 mg/kg/day Admission Discharge
  • 63. tRNA synthetase syndrome with predominant interstitial lung disease Seconday Sjogren's syndrome Final Diagnosis | ADMISSION VGHTC_IMRH - s/p partial thyroidectomy in 1999
 - without medication of eltroxin Thyroid Goiter - Euthyroidism status - SSA +
 - Schimer test + - EJ +++, RO-52 +++
 - HRCT
  • 65. Interstitial Pneumonia with Autoimmune Features | CLASSIFICATION CRITERIA
  • 66. Morphologic | HRCT - NSIP W/ OP OVERLAP | UNEXPLAINED PFT VGHTC_IMRH
  • 67. Clinical | DISTAL DIGITAL FISSURING VGHTC_IMRH
  • 68. Serologic | RF ≥ 2X ULN | ANTI-RO | ANTI-TRNA SYNTHETASE (EJ) VGHTC_IMRH
  • 69. Laboratory Results No Elevation of Myositis / Inflammatory Markers
  • 70. Immunologic Panel Autoantibody RF ANA dsDNA RO-52 SSB Ab RO-60 30.5 IU/mL Positive, 1:160
 Nucleoplasm - Fine speckled 58.1 WHO units/mL Positive
 63 EliA U/ml Negative
 < 0.3 EliA U/ml Positive
 33 EliA U/ml Complement C3 C4 116.5 mg/dl 23.2 mg/dL Immunoglobin IgG Total IgE 1611 mg/dl 80.4 kU/l Inflammation Index Ferritin ESR 54.5 ng/ml 31 MM/HR Others Cellfree IL-2R 245.4 pg/ml Laboratory Results / 20181228