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Table 2 Immunofluorescence, systemic findings, treatment, and outcome of the patients with BSLE

From: Clusters of clinical and immunologic features in patients with bullous systemic lupus erythematosus: experience from a single-center cohort study in China

No

Age/Sex

IIF

DIF

ELISA

Systemic findings

SLEDAI

SLE characteristic laboratory values

Treatment

Outcome

Anti-BP180 antibody

Anti-BP230 antibody

Anti-type VII collagen antibody

1

M/36

Anti-BMZ antibodies located on the dermal side

No data

Negative

Negative

Negative

AIHA, leukopenia, arthritis, LN, NPSLE, serositis

21

Hypocomplementemia, ANA H1:320, positive anti-dsDNA and anti-rRNP

GC (pulse), CTX, MMF

Lost

2

F/17

Anti-BMZ antibodies located on the dermal side

Negative

Negative

Negative

Positive

LN

4

ANA HS1:80, positive anti-rRNP, anti-Sm and anti-SSA

GC, CsA, HCQ

Lost

3

F/13

Anti-BMZ antibodies located on the dermal side

linear IgG and IgM on BMZ

Negative

Negative

Positive

LN, hemocytopenia

10

Hypocomplementemia, ANA H:1280, positive anti-dsDNA and anti-rRNP

GC, CTX, MMF, HCQ

Improved

4

M/32

Negative

Negative

Negative

Negative

Positive

LN, hemocytopenia, PAH, serositis

25

Hypocomplementemia, ANA H:1280, positive anti-dsDNA

GC (pulse), CTX

Improved

5

F/24

Anti-BMZ antibodies located on the dermal side

No data

Negative

Negative

Positive

LN, hemocytopenia, myocarditis, NPSLE, serositis

19

Hypocomplementemia, ANA S1:80, positive anti-RNP

GC (pulse), HCQ

Lost

6

F/13

Anti-BMZ antibodies located on the dermal side

Linear IgG and IgM on BMZ

Negative

Negative

Positive

LN, hemocytopenia, arthritis, serositis

3

Hypocomplementemia, ANA H1:640, positive anti-dsDNA, anti-SSA and anti-SSB

GC (pulse), CTX

Relapsed

7

F/36

Negative

Linear IgG, IgM, IgA and C3 on BMZ

Negative

Negative

Negative

Arthritis, LN, ILD

6

ANA S1:1280, positive anti-RNP, anti-SSA and anti-SSB

GC (pulse), CTX, HCQ

Improved

8

F/27

Negative

Negative

Negative

Negative

Positive

LN, IPO, hemocytopenia

10

Hypocomplementemia, ANA H1:320, positive anti-dsDNA, anti-SSA and anti-SSB

GC (pulse*2), CTX, FK506, HCQ

Improved

9

F/28

Anti-BMZ antibodies located on the dermal side

No data

Negative

Negative

Negative

LN, hemocytopenia, arthritis, serositis

20

Hypocomplementemia, ANA H1:160, positive anti-dsDNA, ACL, and LA

GC (pulse), CTX, HCQ

Improved

10

F/22

Anti-BMZ antibodies located on the dermal side

No data

Negative

Negative

Positive

LN, serositis

12

Hypocomplementemia, ANA HS1:320, positive anti-dsDNA, anti-Sm, anti-RNP and anti-SSA

GC, MMF, HCQ

Improved

11

F/56

Negative

Negative

Negative

Negative

Positive

Hemocytopenia, arthritis,

8

Hypocomplementemia, ANA S1:160, positive anti-SSA

GC, TGP

Relapsed

12

F/7

Anti-BMZ antibodies located on the dermal side

Linear IgG, IgM, and IgA on BMZ

Negative

Negative

Negative

Hemocytopenia, arthritis, LN, ILD, NPSLE

18

Hypocomplementemia, ANA cytoplasmic 1:320, positive anti-dsDNA and anti-rRNP

GC, FK506, HCQ

Improved

  1. IIF indirect immunofluorescence, DIF direct immunofluorescence, BMZ basement membrane zone, ELISA enzyme-linked immunosorbent assay, SLEDAI SLE disease activity index, AIHA: autoimmune hemolytic anemia, LN lupus nephritis, NPSLE neuropsychiatric systemic lupus erythematosus, IPO intestinal pseudo-obstruction, ILD interstitial lung disease, GC glucocorticoid, CTX cyclophosphamide, MMF mycophenolate mofetil, HCQ hydroxychloroquine, TGP total glucosides of peony, FK506 tacrolimus, CsA cyclosporine A, Anti-dsDNA anti-double stranded DNA antibody, Anti-Sm anti-Smith antibody, Anti-SSA anti-SSA antibody, Anti-SSB anti-SSB antibody, Anti-RNP anti-u1 small-nuclear RNA–protein antibody, Anti-rRNP antiribosomal RNA–protein antibody, LA lupus anticoagulant, ACL anti cardiolipin antibody