An autoantibody to Cl-inhibitor recognizes the reactive center of the inhibitor

Donaldson, Virginia H.; Wagner, Constance; Davis, Alvin E.

doi:10.1016/s0022-2143(96)90083-9

Cited by 13 publications

(3 citation statements)

References 10 publications

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“…Pathophysiology suggests that bradykinin‐targeted drugs, licensed to treat HAE due to C1 inhibitor deficiency, could be effective to reverse symptoms in ACEI‐AAE . Due to the lack of efficacy of corticosteroids and epinephrine, some of them have been used off label in ACEI‐AAE.…”

Section: Acquired Angioedema Related To Angiotensin‐converting Enzymementioning

confidence: 99%

“…Acquired angioedema with C1 inhibitor deficiency can be caused by autoantibodies neutralizing C1‐INH function ‐binding epitopes mapped around its reactive center . Although initially identified as an independent form of acquired C1‐INH deficiency, large case series demonstrated that C1‐INH‐AAE with autoantibodies and with lymphoproliferative diseases largely overlaps and should be considered the same disease .…”

Section: Acquired Angioedema With C1 Inhibitor Deficiencymentioning

confidence: 99%

See 1 more Smart Citation

Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group

Cicardi

Aberer

Banerji

et al. 2014

Allergy

620

694

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Angioedema is defined as localized and self-limiting edema of the subcutaneous and submucosal tissue, due to a temporary increase in vascular permeability caused by the release of vasoactive mediator(s). When angioedema recurs without significant wheals, the patient should be diagnosed to have angioedema as a distinct disease. In the absence of accepted classification, different types of angioedema are not uniquely identified. For this reason, the European Academy of Allergy and Clinical Immunology gave its patronage to a consensus conference aimed at classifying angioedema. Four types of acquired and three types of hereditary angioedema were identified as separate forms from the analysis of the literature and were presented in detail at the meeting. Here, we summarize the analysis of the data and the resulting classification of angioedema.

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Section: Acquired Angioedema Related To Angiotensin‐converting Enzymementioning

confidence: 99%

Section: Acquired Angioedema With C1 Inhibitor Deficiencymentioning

confidence: 99%

Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group

Cicardi

Aberer

Banerji

et al. 2014

Allergy

620

694

View full text Add to dashboard Cite

show abstract

“…Some evidence indicates that these autoantibodies bind to epitopes located near the reactive centre of the inhibitor molecule. [18][19][20] In the opinion of Mandle et al, 18 these autoantibodies sterically inhibit the interaction between C1s and C1-INH, through binding to the reactive centre. Therefore, C1s are not inhibited and this lack of inhibition enhances the action of the complement system.…”

Section: Discussionmentioning

confidence: 99%

C1-inhibitor autoantibodies in SLE

Mészáros

Füst

Farkas

et al. 2010

Lupus

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The presence of anti-C1-inhibitor (anti-C1-INH) autoantibodies is a hallmark of acquired C1-inhibitor deficiency. However, only scarce data are available on their prevalence, diagnostic value, and/or significance in systemic lupus erythematosus (SLE). In a multicentre study, we determined the levels of autoantibodies to C1-inhibitor in sera from 202 patients with SLE and 134 healthy controls. Additional clinical and laboratory parameters, such as organ involvement, as well as anti-C1q, anti-double-stranded DNA antibody, erythrocyte sedimentation rate, C-reactive protein, C3 and C4 serum complement levels have been studied in patients. The level of anti-C1-INH IgG was significantly higher (p = 0.034) in SLE patients, than in the controls. A high anti-C1-INH level of > or =0.4 U/ml (mean of controls + 2 SD) was found in 17% of the patients, but in only 4% of the controls (p = 0.0003). The SLEDAI score was significantly higher (p = 0.048) and the duration of SLE was significantly longer (p = 0.0004) among patients with elevated anti-C1-INH levels compared with patients without this autoantibody (median disease duration 8 vs. 17 years, respectively). Anti-C1-INH level was not correlated with any other laboratory parameter or organ manifestation of the disease. These findings indicate that the anti-C1-INH level is higher in SLE patients than in healthy controls and furthermore, the anti-C1-INH level correlates with the duration and activity of the disease.

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Angioedema associated with C1 inhibitor deficiency

Laurent

Guinnepain

1999

Clinic Rev Allerg Immunol

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An autoantibody to Cl-inhibitor recognizes the reactive center of the inhibitor

Cited by 13 publications

References 10 publications

Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group

Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group

C1-inhibitor autoantibodies in SLE

Angioedema associated with C1 inhibitor deficiency

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