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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.