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Table 2 Clinical features and investigations in Schnitzler’s syndrome

From: Schnitzler’s syndrome: lessons from 281 cases

 

Percentage

Number of cases

Clinical features

  

Chronic urticaria

100%

281

Pruritus

21%

58

Intermittent fever

72%

203

Arthralgia, rarely overt arthritis

68%

192

Bone pain

55%

155

Weight loss

16%

45

Angioedema

8%

22

Lymphadenopathy

26%

72

Hepatomegaly

9%

24

Splenomegaly

6%

17

Neuropathy

7%

20

Laboratory investigations

  

Elevated ESR/CRP

97%

174/179

Leukocytosis

75%

115/153

Anemia

63%

62/98

paraprotein

100%

281

  IgMκ total

85%

232

  IgMκ monoclonal

79%

222

  IgMλ monoclonal

8%

22

  IgGκ monoclonal

5%

13

  IgGλ monoclonal

1%

4

  IgM, type not specified

2%

7

  Ig, type not specified

1%

3

  IgMκ & IgMλ

1%

3

  IgMκ & IgMκ & IgMλ

0,4%

1

  IgMκ & IgGκ

0,4%

1

  IgMλ & IgGκ

0,4%

1

  IgMκ & IgAκ

0,4%

1

  IgMκ & IgAλ

0,4%

1

  IgMκ > IgGκ > undetectable

0,4%

1

  IgMκ > polyclonal

0,4%

1

  κ light chain subtype

89%

249/281

Bence Jones proteins

23%

14/62

Skin biopsies

  

dominant histological features

100%

207

Neutrophils

50%

104

Lymphocytes +/− macrophages

5%

11

Macrophages

5%

11

Vasculitis

20%

41

Otherwise (mostly unspecified, no vasculitis)

19%

40

immune deposits

100%

83

  None

70%

58

  Present

30%

25

  IgM

23%

19

  IgG

2%

2

  C3

14%

12

Skeletal examinations

  

conventional radiographs

100%

146

  Normal

57%

83

  Hyperostosis

39%

57

osteolysis

1%

2

  Periostal reaction

0,7%

1

  Arthritis

0,7%

1

  Abnormal, not specified

1%

2

scintigraphy

100%

97

  Normal

13%

13

  Increased uptake

85%

82

  Infarctions tibiae

1%

1

  Osteomyelitis

1%

1

bone biopsy

100%

14

  Normal

43%

6

  Sclerosis

29%

4

  Increased osteoblast & -clast activity

14%

2

  Inflammation

7%

1

  Aspecific

7%

1

Bone marrow aspirates or biopsies

100%

164

  Normal

63%

104

  Malignancy*

21%**

34

  Plasmacytosis

9%

15

  Otherwise (mostly aspecific)

7%

11

  1. Percentages and numbers of patients in which clinical features, laboratory investigations, histological features of skin biopsies, skeletal examinations and bone marrow aspirates were reported.
  2. *Specified in Table 4.
  3. **Likely to be an overestimation, as in multiple reports, bone marrow aspirates were not mentioned.
  4. The bold numbers indicate the number of cases in which it was reported.
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Clinical and Translational Allergy

ISSN: 2045-7022

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