No.

1

2

3

4

5

6

7

8

9

10

11

12

age/gender

53/F

61/F

58/M

27/M

65/M

58/F

36/F

47/F

32/F

35/F

63/F

61/F

Fever

(−)

(+)

(−)

(−)

(−)

(−)

(−)

(+)

(+)

(−)

(−)

(−)

Skin manifestation

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

Arthritis/arthralgia

(+)

(−)

(+)

(−)

(+)

(−)

(+)

(−)

(−)

(−)

(+)

(−)

Muscle weakness

(−)

(−)

(−)

(−)

(−)

(−)

(+)

(−)

(−)

(−)

(−)

(−)

Raynaud’s phenomenon

(+)

(+)

(+)

(−)

(−)

(+)

(−)

(+)

(−)

(−)

(−)

(+)

Sclerodactyly

(−)

(−)

(−)

(−)

(−)

(+)

(−)

(+)

(−)

(−)

(−)

(−)

Sicca syndrome

(−)

(+)

(−)

(−)

(−)

(+)

(−)

(−)

(−)

(−)

(+)

(−)

ANA

(+)

(−)

(−)

(−)

(+)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

RF

(−)

(+)

(+)

(−)

(−)

(−)

(+)

(−)

(−)

(−)

(+)

(−)

Anti-ARS

EJ

PL−12

EJ

OJ

KS

KS

KS

PL−12

OJ

PL−12

EJ

KS

Other antibodies

(−)

SSA

(−)

(−)

(−)

(−)

(−)

(−)

(−)

SSA

(−)

(−)

%VC (%)

78

65

78

74

67

85

35

56

36

74

86

n.d.

DLco/VA ml/min/Torr/l)

5.2

5.0

3.0

5.9

4.3

4.2

n.a.

4.5

4.1

4.2

4.9

n.a.

KL-6 (U/ml)

231

1620

957

1410

1470

1520

n.a.

742

1700

7080

2040

1230

Pathological findings

OP

NSIP

DIP

UIP

n.a.

LIP

n.d.

UIP

NSIP

n.a.

NSIP

n.d.

Uptake in Gallium scintigram

(+)

(+)

(+)

(+)

(+)

(−)

(+)

(−)

n.d.

n.d.

n.d.

n.d.

PSL (mg/day)

(−)

40

60

60

40

(−)

(−)

(−)

50

Pulse/70

60

(−)

Immunosuppressant

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(+)

(−)

(−)

Outcome

nca

slpb

nc

impc

slp

nc

slp

nc

imp

imp

imp

nc