Table 2 Characteristics of the six anti-SRP IMNM overlap SS patients

Demographics

 Sex (Male/Female)

M

F

F

F

M

F

 Age at diagosis of SS (years)

18

26

35

53

42

61

 Disease duration (months)

12

2

4

3

72

3

Diagosis criteria for SS

 Dry eye

-

-

-

-

-

 + 

 Dry mouth

-

-

-

 + 

-

-

 Shirmer's test

-

NA

NA

NA

-

 + 

 Decreased salivary flow

-

-

-

-

 + 

 + 

 Parotid swelling

-

-

-

-

-

-

 Pathologic salivary gland biopsy ( focal lymphocytic sialad

 + 

 + 

 + 

 + 

 + 

 + 

 FS

1

2

1

1

2

2

 Anti-SSA

 + 

 + 

 + 

 + 

 + 

 + 

 Anti-SSB

 + 

-

-

-

 + 

-

 Anti-SSA/Ro-52

-

 + 

-

 + 

 + 

 + 

Clinical manifestation

 Upper promixal(MRC ≤ 3)

-

-

-

-

-

 + 

 Upper distal(MRC ≤ 3)

-

-

-

-

-

-

 Lower promixal(MRC ≤ 3)

-

-

 + 

 + 

-

 + 

 Lower distal(MRC ≤ 3)

-

-

-

-

-

 + 

 MMT

230

184

240

206

235

140

 DAS

2

6

2

3

1

7

 MYOCAT

16

15

5

5

5

21

 Atrophy

-

-

-

-

-

-

 Dyspnea

-

-

-

-

-

-

 Dysphagia

-

-

-

-

-

-

 Myalgia

 + 

-

-

 + 

-

 + 

 Thyroid dysfunction

 + 

-

-

 + 

-

 + 

 Lupus Anticoagulant

-

-

-

-

-

-

 Cutaneous

-

-

-

-

-

 + 

 Statin exposure

-

-

-

-

-

-

 Smoking

-

-

-

-

-

-

 Drinking

-

-

-

-

-

-

 Diabete

-

-

-

-

-

-

 Hypertension

-

-

-

-

 + 

-

 Renal

-

-

-

-

-

-

 B cell lymphoma

-

-

-

-

-

-

 Central nervous system

-

-

-

-

-

-

 Peripheral nervous system

-

-

-

-

-

-

 Arthritis

-

-

-

-

-

-

 Pulmonary

-

 + 

-

 + 

-

-

 Cardiac abnormality

 + 

-

-

 + 

 + 

-

 Vascular

-

-

-

-

-

-

 Haematological

-

-

-

-

-

-

 Raynaud's phenomena

-

-

-

-

-

-

Examination

 ANCA

-

-

-

-

-

-

 ANA Titer

 + 

 + 

 + 

 + 

-

 + 

 ElevatedCK level (U/l)

8419

7853

9516

321

7319

4000

 Elevated LDH level (U/l)

1621

410

377

454

1306

793

 AST (U/l)

130

148

267

25

136

121

 ALT (U/l)

127

144

172

25

368

218

 Myoglobin(ng/ml)

729.6

672.6

862.9

 > 1200

-

894

 CK-MB(ng/ml)

157

64.3

968

115.4

-

-

 TnI(pg/ml)

12

19

-

-

22

-

 NI-PRO-BNP(pg/ml)

-

-

-

290

-

-

 ESR(mm/H)

-

-

-

16

25

-

 hsCRP (mg/L)

NA

0.3

0.2

NA

5

8

 Alb(g/L)

44.2

33.7

33.7

44.7

32.5

39.7

 Glb(g/L)

24.7

34.2

NA

27.9

23

23.8

 IgA(g/L)

3.5

2.3

NA

1.69

NA

2.44

 IgG(g/L)

15

13

NA

13.4

NA

13.9

 IgM(g/L)

1.5

0.89

NA

2.22

NA

0.84

 C3(g/L)

1.1

0.69

NA

0.98

NA

0.93

 C4(g/L)

0.25

0.16

NA

0.18

NA

0.23

Pathological examination

 Labial glands biopsy ( focal lymphocytic sialadenitis with a

 + 

 + 

 + 

 + 

 + 

 + 

Treatment outcome

 Immunomodulatory treatment

RTX

TAC,PNL

TAC,PNL

MP

TAC,PNL

TAC,PNL

 Follow-up(months)

72

8

6

36

120

36

 Dry eye

-

-

-

-

-

-

 Dry mouth

-

-

-

-

-

 + 

 No improvement

-

-

-

-

-

-

 improvement

 + 

-

 + 

 + 

 + 

 + 

 Marked improvement

-

 + 

-

-

-

-

 Clinical relapse

 + 

-

-

-

-

-