Abstract
Background and Objectives. Autoantibodies inactivating the von Willebrand factor (VWF) cleaving protease, ADAMTS-13, are among the most frequent causes of thrombotic thrombocytopenic purpura (TTP). We evaluated whether or not ADAMTS-13 deficiency and autoantibodies inactivating the protease prevalent in patients with the prototypic autoimmune diseases systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Design and Methods. We measured, in parallel, the protease and VWF antigen (VWF:Ag) in 123 patients, 36 of whom had SLE and 87 of whom had SSc. In 14 patients with either disease who had low plasma protease levels (below 40%) we also looked for anti-ADAMTS-13 inactivating antibodies. Results. ADAMTS-13 levels were significantly lower in SLE (p=0.0013) and in SSc (p=0.0002) than in normal controls. No anti-ADAMTS activity was measurable in patients with low ADAMTS-13 levels. VWF: Ag was high in both SLE and SSc (p=0.001). Interpretation and Conclusions. Systemic connective tissue diseases are other conditions besides TTP that are associated in some instances with low but detectable levels of ADAMTS-13. Autoantibodies inactivating protease activity are not the cause of the low plasma levels of ADAMTS-13.
| Original language | English |
|---|---|
| Pages (from-to) | 914-918 |
| Number of pages | 5 |
| Journal | Haematologica |
| Volume | 88 |
| Issue number | 8 |
| Publication status | Published - Aug 1 2003 |
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Keywords
- ADAMTS-13
- Systemic lupus erythematosus
- Systemic sclerosis
- Thrombotic microangiopathies
- Von Willebrand factor V
ASJC Scopus subject areas
- Hematology
Cite this
Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis). / Mannucci, Pier Mannuccio; Vanoli, Massimo; Forza, Ileana; Canciani, Maria Teresa; Scorza, Raffaella.
In: Haematologica, Vol. 88, No. 8, 01.08.2003, p. 914-918.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis)
AU - Mannucci, Pier Mannuccio
AU - Vanoli, Massimo
AU - Forza, Ileana
AU - Canciani, Maria Teresa
AU - Scorza, Raffaella
PY - 2003/8/1
Y1 - 2003/8/1
N2 - Background and Objectives. Autoantibodies inactivating the von Willebrand factor (VWF) cleaving protease, ADAMTS-13, are among the most frequent causes of thrombotic thrombocytopenic purpura (TTP). We evaluated whether or not ADAMTS-13 deficiency and autoantibodies inactivating the protease prevalent in patients with the prototypic autoimmune diseases systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Design and Methods. We measured, in parallel, the protease and VWF antigen (VWF:Ag) in 123 patients, 36 of whom had SLE and 87 of whom had SSc. In 14 patients with either disease who had low plasma protease levels (below 40%) we also looked for anti-ADAMTS-13 inactivating antibodies. Results. ADAMTS-13 levels were significantly lower in SLE (p=0.0013) and in SSc (p=0.0002) than in normal controls. No anti-ADAMTS activity was measurable in patients with low ADAMTS-13 levels. VWF: Ag was high in both SLE and SSc (p=0.001). Interpretation and Conclusions. Systemic connective tissue diseases are other conditions besides TTP that are associated in some instances with low but detectable levels of ADAMTS-13. Autoantibodies inactivating protease activity are not the cause of the low plasma levels of ADAMTS-13.
AB - Background and Objectives. Autoantibodies inactivating the von Willebrand factor (VWF) cleaving protease, ADAMTS-13, are among the most frequent causes of thrombotic thrombocytopenic purpura (TTP). We evaluated whether or not ADAMTS-13 deficiency and autoantibodies inactivating the protease prevalent in patients with the prototypic autoimmune diseases systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Design and Methods. We measured, in parallel, the protease and VWF antigen (VWF:Ag) in 123 patients, 36 of whom had SLE and 87 of whom had SSc. In 14 patients with either disease who had low plasma protease levels (below 40%) we also looked for anti-ADAMTS-13 inactivating antibodies. Results. ADAMTS-13 levels were significantly lower in SLE (p=0.0013) and in SSc (p=0.0002) than in normal controls. No anti-ADAMTS activity was measurable in patients with low ADAMTS-13 levels. VWF: Ag was high in both SLE and SSc (p=0.001). Interpretation and Conclusions. Systemic connective tissue diseases are other conditions besides TTP that are associated in some instances with low but detectable levels of ADAMTS-13. Autoantibodies inactivating protease activity are not the cause of the low plasma levels of ADAMTS-13.
KW - ADAMTS-13
KW - Systemic lupus erythematosus
KW - Systemic sclerosis
KW - Thrombotic microangiopathies
KW - Von Willebrand factor V
UR - http://www.scopus.com/inward/record.url?scp=0141649437&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0141649437&partnerID=8YFLogxK
M3 - Article
VL - 88
SP - 914
EP - 918
JO - Haematologica
JF - Haematologica
SN - 0390-6078
IS - 8
ER -