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Table 1 (abstract P2096). See text for description

From: 10th Congress of International Society of Systemic Auto-Inflammatory Diseases (ISSAID)

No

S

Age

Trigger

Disease

Therapy

Outcome

Observations

1.

F

72

Resp. Virus

IIM

life support (ICU); MP pulses; cyclosporine; immunoglobulins

survival

ARF; hypoC4

Atypical rash

2.

F

56

Acute cholecystitis

PG

Life support (ICU); immunoglobulins; MP, CyA

survival

 

3.

F

58

viral

RA

MP, CyA, MTX

survival

 

4.

F

46

?

SLE/SSj (Ro52)

MP, CyA

survival

Evolved to Systemic sclerosis

5.

F

49

Q fever

SLE/SSj (Ro52)

cryoglobulinemia

Life support (ICU); plasma exchange; MP, CyA

survival

TMHA; hypoC3, C4

6.

M

23

?

PAN

MP, CyA

survival

 

7.

F

25

?

SLE

MP, CyA

survival

 

8.

F

57

Resp. virus

inflammatory idiopathic myopathy

MP pulses; CyA, then Aza

survival

Atypical rash

  1. Legend: ARF- acute respiratory failure, Aza- azathioprine, CyA- cyclosporin A,ICU- intensive care unit, IIM= inflammatory idiopathic myopathy, MP- methylprednisolone, PAN- polyarteritis nodosa, PG- pyoderma gangrenosum (systemic), RA-rheumatoid arthritis, SLE- systemic lupus erythematosus, SjS- Sjogren’s syndrome, TMHA- thrombotic microangiopathy