IgA Vasculitis With Underlying Liver Cirrhosis : A French Nationwide Case Series of 20 Patients
Copyright © 2021 by the Journal of Rheumatology..
OBJECTIVE: Immunoglobulin A vasculitis (IgAV) and nephropathy (IgAN) share common immunological mechanisms. Liver cirrhosis is well known to be associated with IgAN. Here, we aimed to describe the presentation and outcome of IgAV patients with underlying cirrhosis.
METHODS: We conducted a French nationwide retrospective study of adult patients presenting with both IgAV and cirrhosis. Baseline characteristics were compared to those of the 260 patients included in the French nationwide IgAV registry (IGAVAS).
RESULTS: Twenty patients were included, and 7 (35%) were female. The mean ± SD age was 62.7 ± 11 years. At baseline, compared with IGAVAS patients, patients with underlying cirrhosis were older (62.7 ± 11 vs 50.1 ± 18, P < 0.01) and displayed more constitutional symptoms (weight loss 25% vs 8%, P = 0.03). Patients with underlying cirrhosis were also more likely to exhibit elevated serum IgA levels (5.6 g/L vs 3.6 g/L, P = 0.02). Cirrhosis and IgAV were diagnosed simultaneously in 12 patients (60%). Cirrhosis was mainly related to alcohol intake (n = 15, 75%), followed by nonalcoholic steato-hepatitis (n = 2), chronic viral hepatitis (n = 1), hemochromatosis (n = 1), and autoimmune hepatitis (n = 1). During follow-up with a median of 17 months (IQR 12-84), 10/13 (77%) exhibited IgAV remission at Month 3. One patient presented a minor relapse. Six patients died, but no deaths were related to IgAV.
CONCLUSION: We report the first case series of IgAV patients with underlining cirrhosis, to our knowledge, which was mainly alcohol related. The liver disease did not seem to affect baseline vasculitis characteristics. Physicians should investigate the existence of liver cirrhosis at IgAV diagnosis, especially in the context of alcohol abuse.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:48 |
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| Enthalten in: |
The Journal of rheumatology - 48(2021), 5 vom: 14. Mai, Seite 735-740 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Elhani, Ines [VerfasserIn] |
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| Links: |
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| Themen: |
Alcohol |
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| Anmerkungen: |
Date Completed 27.09.2021 Date Revised 04.12.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.3899/jrheum.200293 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM313757445 |
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| 100 | 1 | |a Elhani, Ines |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a IgA Vasculitis With Underlying Liver Cirrhosis |b A French Nationwide Case Series of 20 Patients |
| 264 | 1 | |c 2021 | |
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| 500 | |a Date Completed 27.09.2021 | ||
| 500 | |a Date Revised 04.12.2021 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2021 by the Journal of Rheumatology. | ||
| 520 | |a OBJECTIVE: Immunoglobulin A vasculitis (IgAV) and nephropathy (IgAN) share common immunological mechanisms. Liver cirrhosis is well known to be associated with IgAN. Here, we aimed to describe the presentation and outcome of IgAV patients with underlying cirrhosis | ||
| 520 | |a METHODS: We conducted a French nationwide retrospective study of adult patients presenting with both IgAV and cirrhosis. Baseline characteristics were compared to those of the 260 patients included in the French nationwide IgAV registry (IGAVAS) | ||
| 520 | |a RESULTS: Twenty patients were included, and 7 (35%) were female. The mean ± SD age was 62.7 ± 11 years. At baseline, compared with IGAVAS patients, patients with underlying cirrhosis were older (62.7 ± 11 vs 50.1 ± 18, P < 0.01) and displayed more constitutional symptoms (weight loss 25% vs 8%, P = 0.03). Patients with underlying cirrhosis were also more likely to exhibit elevated serum IgA levels (5.6 g/L vs 3.6 g/L, P = 0.02). Cirrhosis and IgAV were diagnosed simultaneously in 12 patients (60%). Cirrhosis was mainly related to alcohol intake (n = 15, 75%), followed by nonalcoholic steato-hepatitis (n = 2), chronic viral hepatitis (n = 1), hemochromatosis (n = 1), and autoimmune hepatitis (n = 1). During follow-up with a median of 17 months (IQR 12-84), 10/13 (77%) exhibited IgAV remission at Month 3. One patient presented a minor relapse. Six patients died, but no deaths were related to IgAV | ||
| 520 | |a CONCLUSION: We report the first case series of IgAV patients with underlining cirrhosis, to our knowledge, which was mainly alcohol related. The liver disease did not seem to affect baseline vasculitis characteristics. Physicians should investigate the existence of liver cirrhosis at IgAV diagnosis, especially in the context of alcohol abuse | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Henoch-Schönlein purpura | |
| 650 | 4 | |a IgA vasculitis | |
| 650 | 4 | |a alcohol | |
| 650 | 4 | |a cirrhosis | |
| 650 | 4 | |a liver | |
| 650 | 7 | |a Immunoglobulin A |2 NLM | |
| 700 | 1 | |a Pillebout, Evangéline |e verfasserin |4 aut | |
| 700 | 1 | |a Terrier, Benjamin |e verfasserin |4 aut | |
| 700 | 1 | |a Hankard, Antoine |e verfasserin |4 aut | |
| 700 | 1 | |a Vrtovsnik, François |e verfasserin |4 aut | |
| 700 | 1 | |a Jourde-Chiche, Noémie |e verfasserin |4 aut | |
| 700 | 1 | |a Greillier, Sophie |e verfasserin |4 aut | |
| 700 | 1 | |a Groh, Matthieu |e verfasserin |4 aut | |
| 700 | 1 | |a Belfeki, Nabil |e verfasserin |4 aut | |
| 700 | 1 | |a Bigot, Adrien |e verfasserin |4 aut | |
| 700 | 1 | |a de Boysson, Hubert |e verfasserin |4 aut | |
| 700 | 1 | |a Pageaux, Georges-Philippe |e verfasserin |4 aut | |
| 700 | 1 | |a Raffray, Loïc |e verfasserin |4 aut | |
| 700 | 1 | |a Urbanski, Geoffrey |e verfasserin |4 aut | |
| 700 | 1 | |a Ollivier, Isabelle |e verfasserin |4 aut | |
| 700 | 1 | |a Maillot, Francois |e verfasserin |4 aut | |
| 700 | 1 | |a Aouba, Achille |e verfasserin |4 aut | |
| 700 | 1 | |a Audemard-Verger, Alexandra |e verfasserin |4 aut | |
| 700 | 0 | |a Alexandra Audemard-Verger on behalf of the French Vasculitis Study Group (FVSG) and the HSPrognosis Group |e verfasserin |4 aut | |
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