PMID- 33215769
OWN - NLM
STAT- MEDLINE
DCOM- 20220217
LR  - 20220217
IS  - 1365-2796 (Electronic)
IS  - 0954-6820 (Linking)
VI  - 289
IP  - 4
DP  - 2021 Apr
TI  - A follow-up survey of patients with acquired angioedema due to C1-inhibitor 
      deficiency.
PG  - 547-558
LID - 10.1111/joim.13182 [doi]
AB  - BACKGROUND: Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a 
      rare form of bradykinin-mediated angioedema. It is diagnosed by complement 
      testing; its treatment consists of the management of angioedema (AE) attacks and 
      of underlying disease. OBJECTIVE: Evaluate the results of the clinical follow-up 
      of patients with C1-INH-AAE. METHODS: Between 1999 and 2020, 3938 patients with 
      angioedema were evaluated, and 17 diagnosed with acquired C1-INH deficiency were 
      followed-up. RESULTS: Mean age of the 17 patients was 61 years at diagnosis. In 
      33%, ACE inhibitors provoked AE attacks. Autoantibodies against C1-INH were 
      detected in 10 patients at diagnosis and in a further patient during follow-up. 
      The AE attacks involved the skin in 70.6%, the upper airways in 41.2% and the 
      tongue/lip in 52.9% of patients. Twelve of the 17 patients had an underlying 
      condition, mainly (n = 11) lymphoproliferative disease. In 10 patients diagnosed 
      with a haematological disorder, AAE symptoms preceded the onset of the latter. 
      One patient has not experienced an AE attack since diagnosis. Twelve patients 
      were treated for angioedema attacks, and 32% of the attacks required acute 
      treatment. PdC1-INH was used to relieve AE attacks, and rituximab for the 
      treatment of underlying disease (in six patients). Six patients had multiple AE 
      attacks before any treatment. The symptom-free period increased in five patients 
      after the on-demand administration of pdC1-INH concentrate and following 
      treatment of the underlying disease in two patients. CONCLUSION: Early diagnosis 
      of C1-INH-AAE and underlying disease is indispensable to reduce disease burden by 
      introducing appropriate, individualized treatment and regular follow-up.
CI  - (c) 2020 The Association for the Publication of the Journal of Internal Medicine.
FAU - Polai, Zs
AU  - Polai Z
AD  - From the, Hungarian Angioedema Center of Reference and Excellence, Department of 
      Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
FAU - Balla, Zs
AU  - Balla Z
AD  - From the, Hungarian Angioedema Center of Reference and Excellence, Department of 
      Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
FAU - Andrasi, N
AU  - Andrasi N
AD  - From the, Hungarian Angioedema Center of Reference and Excellence, Department of 
      Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
AD  - 2nd Department of Pediatrics, Semmelweis University, Budapest, Hungary.
FAU - Kohalmi, K V
AU  - Kohalmi KV
AD  - From the, Hungarian Angioedema Center of Reference and Excellence, Department of 
      Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
AD  - Buda Hospital of the Hospitaller Order of Saint John of God, Rheumatology Center, 
      Budapest, Hungary.
FAU - Temesszentandrasi, Gy
AU  - Temesszentandrasi G
AD  - Department of Internal Medicine and Haematology, Semmelweis University, Budapest, 
      Hungary.
FAU - Benedek, Sz
AU  - Benedek S
AD  - Department of Internal Medicine and Haematology, Semmelweis University, Budapest, 
      Hungary.
FAU - Varga, L
AU  - Varga L
AD  - From the, Hungarian Angioedema Center of Reference and Excellence, Department of 
      Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
FAU - Farkas, H
AU  - Farkas H
AUID- ORCID: 0000-0003-2929-1721
AD  - From the, Hungarian Angioedema Center of Reference and Excellence, Department of 
      Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20201120
PL  - England
TA  - J Intern Med
JT  - Journal of internal medicine
JID - 8904841
RN  - 0 (Complement C1 Inhibitor Protein)
RN  - Acquired angioedema
SB  - IM
MH  - *Angioedema/diagnosis/drug therapy/etiology
MH  - *Angioedemas, Hereditary/diagnosis/drug therapy
MH  - Complement C1 Inhibitor Protein
MH  - Follow-Up Studies
MH  - Humans
MH  - Middle Aged
OTO - NOTNLM
OT  - C1 inhibitor deficiency
OT  - acquired angioedema
OT  - angiotensin-converting enzyme inhibitors
OT  - lymphoproliferative disease
OT  - rituximab
EDAT- 2020/11/21 06:00
MHDA- 2022/02/19 06:00
CRDT- 2020/11/20 08:40
PHST- 2020/09/02 00:00 [revised]
PHST- 2020/06/15 00:00 [received]
PHST- 2020/09/04 00:00 [accepted]
PHST- 2020/11/21 06:00 [pubmed]
PHST- 2022/02/19 06:00 [medline]
PHST- 2020/11/20 08:40 [entrez]
AID - 10.1111/joim.13182 [doi]
PST - ppublish
SO  - J Intern Med. 2021 Apr;289(4):547-558. doi: 10.1111/joim.13182. Epub 2020 Nov 20.