First Case of Acquired Functional CI INH Deficiency: Association with Angioedema during Churg and Strauss Vasculitis

Pasquali, Jean‐Louis; Christmann, D.; Modert, Fernand; Belval, P. Challan; Storck, D; Hauptmann, G.

doi:10.1159/000233560

Cited by 12 publications

(5 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In the variant form of the disease, immunochemically detectable con centrations of Cl-INH are normal or ele vated, but a dysfunctional mutant protein is synthesized [12]. A reduction of functional Cl-INH may also be acquired; such condi tions may or may not be associated with reduced antigen levels [13,14], Furthermore, functional inadequacy of Cl-INH can be transient without any decrease in the level of Cl-INH [15], Thus the unequivocal diagno sis of all Cl-INH deficiency stages is based on the demonstration of a functionally inade quate Cl-INH.…”

Section: Introductionmentioning

confidence: 99%

Quantification of Cl-Inhibitor Functional Activities by Immunodiffusion Assay in Plasma of Patients with Hereditary Angioedema - Evidence of a Functionally Critical Level of Cl-Inhibitor Concentration

Späth¹,

Wüthrich²,

Butler³

1984

Complement

View full text Add to dashboard Cite

The relationship of C1 -inhibitor (C1 -INH) concentration and apparent functional activity was investigated in 111 plasma samples from 21 patients with the common form of hereditary angioedema (HAE). Functional C1 -INH was analyzed by means of a modified version of immunodiffusion assay. Down to a C1-INH level of approximately 0.075 g/1 (38% of normal) apparent C1-INH functions were found within the normal range, while below this level functional adequacy of C1-INH could no longer be ascertained. When C4 concentrations, considered to reflect approximately functional C1-INH, were related to C1- INH antigen levels of individual samples, a relationship emerged which was identical to that between C1-INH concentration and apparent function. No attacks of edema could be associated with C1 -INH concentrations above 0.07 5 g/1, while it was possible to associate attacks with concentrations below this level. In experiments where patient plasma and normal plasma were mixed in various ratios or where HAE plasma was replaced by purified C1-INH, an increase in C1-INH antigen to concentrations of 0.06-0.08 g/1 was followed by a sharp rise in apparent functions to normal values. The rise of functional C1-INH became moderate when C1-INH antigen further increased. The results supported the idea of a functionally critical level of C1 -INH in the common form of HAE.

show abstract

Section: Introductionmentioning

confidence: 99%

Quantification of Cl-Inhibitor Functional Activities by Immunodiffusion Assay in Plasma of Patients with Hereditary Angioedema - Evidence of a Functionally Critical Level of Cl-Inhibitor Concentration

Späth¹,

Wüthrich²,

Butler³

1984

Complement

View full text Add to dashboard Cite

show abstract

“…Due to the low C4 level, we considered type I acquired angioedema (AAE-I) in differential diagnosis. AAE-I has mainly been reported in patients with autoimmune diseases, such as systemic lupus erythematosus and eosinophilic granulomatosis (6)(7)(8). However, in this patient the quantitative and functional analyses of C1-inhibitor and C1q levels were normal, and AAE was excluded from consideration.…”

Section: Discussionmentioning

confidence: 78%

Chronic peripheral edema in a patient with rheumatoid arthritis

Breznik

Dai

Marovt

2018

Acta Dermatovenerologica Alpina Pannonica Et Adriatica

View full text Add to dashboard Cite

Chronic peripheral edema is a very common yet underestimated problem. It is of utmost importance to investigate various possible causes and understand the pathophysiological mechanisms of chronic peripheral edema in order to determine the optimal treatment. This case report presents the case of a polymorbid patient with a deformity-causing type of rheumatoid arthritis and a 1-year history of progressive symmetrical peripheral edema. Based on an extensive diagnostic investigation, the patient was diagnosed with a combined form of chronic peripheral edema comprised of true lymphedema, which was confirmed by lymphoscintigraphy, and filtration edema due to muscular inactivity. Edema as a side effect of tocilizumab was also suspected.

show abstract

“…Das AAE ist beschrieben worden im Zusammenhang mit autoimmunen, lymphoproliferativen und malignen Erkrankungen, mit Infektionen und Vaskulitiden (u. a. Churg-Strauss-und leukozytoklastische Vaskulitis; [6,7,8]). In der Regel betrifft das AAE Erwachsene oder ältere Patienten [9].…”

Section: Diskussionunclassified

Gesichtsödem als Frühsymptom einer Riesenzellarteriitis

et al. 2010

View full text Add to dashboard Cite

Giant cell arteritis (GCA) is a chronic granulomatous vasculitis of unknown etiology occurring in the elderly. New-onset headache, scalp tenderness, jaw claudication, temporal artery abnormalities on physical examination, visual symptoms and associated polymyalgia rheumatica represent the most typical and frequent features of the disease. However, facial edema is being more commonly recognized as a presenting symptom that may herald the disease. We present a case with facial edema as initial symptom and discuss if this rare symptom of GCA is due to hereditary or acquired angioedema.

show abstract

First Case of Acquired Functional CI INH Deficiency: Association with Angioedema during Churg and Strauss Vasculitis

Cited by 12 publications

References 4 publications

Quantification of Cl-Inhibitor Functional Activities by Immunodiffusion Assay in Plasma of Patients with Hereditary Angioedema - Evidence of a Functionally Critical Level of Cl-Inhibitor Concentration

Quantification of Cl-Inhibitor Functional Activities by Immunodiffusion Assay in Plasma of Patients with Hereditary Angioedema - Evidence of a Functionally Critical Level of Cl-Inhibitor Concentration

Chronic peripheral edema in a patient with rheumatoid arthritis

Gesichtsödem als Frühsymptom einer Riesenzellarteriitis

Contact Info

Product

Resources

About