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MCID: IDP041
MIFTS: 30

Idiopathic Recurrent Pericarditis

Categories: Bone diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Idiopathic Recurrent Pericarditis

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MalaCards integrated aliases for Idiopathic Recurrent Pericarditis:

Name: Idiopathic Recurrent Pericarditis 58
Idiopathic Relapsing Pericarditis 58

Characteristics:

Orphanet epidemiological data:

58
idiopathic recurrent pericarditis
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Bone diseases
See all MalaCards categories (disease lists)
ICD10: 33
Orphanet: 58  
Rare systemic and rhumatological diseases


External Ids:

ICD10 via Orphanet 33 I09.2
Orphanet 58 ORPHA251307
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Summaries for Idiopathic Recurrent Pericarditis

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MalaCards based summary : Idiopathic Recurrent Pericarditis, also known as idiopathic relapsing pericarditis, is related to familial mediterranean fever and periodic fever, familial, autosomal dominant. An important gene associated with Idiopathic Recurrent Pericarditis is TNFRSF1A (TNF Receptor Superfamily Member 1A), and among its related pathways/superpathways are Common Cytokine Receptor Gamma-Chain Family Signaling Pathways and Circadian rythm related genes. The drugs Antirheumatic Agents and Interleukin 1 Receptor Antagonist Protein have been mentioned in the context of this disorder. Affiliated tissues include heart and myeloid, and related phenotypes are homeostasis/metabolism and immune system

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Related Diseases for Idiopathic Recurrent Pericarditis

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Diseases related to Idiopathic Recurrent Pericarditis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 88)
# Related Disease Score Top Affiliating Genes
1 familial mediterranean fever 30.2 TNFRSF1A MEFV
2 periodic fever, familial, autosomal dominant 30.1 TNFRSF1A MEFV
3 hereditary periodic fever syndrome 30.0 TNFRSF1A MEFV
4 pericarditis 29.9 TNFRSF1A RETN MEFV LEP ADIPOQ
5 brucellosis 10.2
6 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
7 constrictive pericarditis 10.1
8 nontuberculous mycobacterial lung disease 10.1 LEP ADIPOQ
9 inflammatory myopathy with abundant macrophages 10.1 TNFRSF1A MEFV
10 intermittent hydrarthrosis 10.1 TNFRSF1A MEFV
11 abdominal obesity-metabolic syndrome quantitative trait locus 2 10.0 LEP ADIPOQ
12 complete generalized lipodystrophy 10.0 LEP ADIPOQ
13 mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome 10.0 LEP ADIPOQ
14 hypothalamic obesity 10.0 RETN LEP
15 adult syndrome 10.0 LEP ADIPOQ
16 palindromic rheumatism 10.0 TNFRSF1A MEFV
17 cervical adenitis 10.0 TNFRSF1A MEFV
18 wells syndrome 10.0 TNFRSF1A MEFV
19 lipodystrophy, familial partial, type 2 10.0 LEP ADIPOQ
20 relapsing fever 10.0 TNFRSF1A MEFV
21 mevalonic aciduria 10.0 TNFRSF1A MEFV
22 adult-onset still's disease 10.0 TNFRSF1A MEFV
23 acanthosis nigricans 10.0 LEP ADIPOQ
24 intracranial hypertension 10.0 RETN ADIPOQ
25 cinca syndrome 10.0 TNFRSF1A MEFV
26 severe pre-eclampsia 10.0 LEP ADIPOQ
27 muckle-wells syndrome 9.9 TNFRSF1A MEFV
28 eating disorder 9.9 LEP ADIPOQ
29 scleroderma, familial progressive 9.9
30 sjogren syndrome 9.9
31 psoriatic arthritis 9.9
32 hemopericardium 9.9
33 cardiac tamponade 9.9
34 pericardial effusion 9.9
35 hellp syndrome 9.9
36 mixed connective tissue disease 9.9
37 connective tissue disease 9.9
38 polyarteritis nodosa 9.9
39 peripheral artery disease 9.9 RETN LEP
40 congenital generalized lipodystrophy 9.9 LEP ADIPOQ
41 uremia 9.9 RETN LEP
42 familial hyperlipidemia 9.9 LEP ADIPOQ
43 pellagra 9.9 NAMPT ADIPOQ
44 lipid storage disease 9.8 LEP ADIPOQ
45 obesity-hypoventilation syndrome 9.8 RETN LEP ADIPOQ
46 acquired generalized lipodystrophy 9.8 RETN LEP ADIPOQ
47 maturity-onset diabetes of the young, type 1 9.8 RETN LEP ADIPOQ
48 apnea, obstructive sleep 9.8 RETN LEP ADIPOQ
49 familial partial lipodystrophy 9.8 RETN LEP ADIPOQ
50 nutritional deficiency disease 9.8 RETN LEP ADIPOQ

Graphical network of the top 20 diseases related to Idiopathic Recurrent Pericarditis:



Diseases related to Idiopathic Recurrent Pericarditis
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Symptoms & Phenotypes for Idiopathic Recurrent Pericarditis

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MGI Mouse Phenotypes related to Idiopathic Recurrent Pericarditis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.73 ADIPOQ LEP MEFV NAMPT RETN TNFRSF1A
2 immune system MP:0005387 9.63 ADIPOQ LEP MEFV NAMPT RETN TNFRSF1A
3 adipose tissue MP:0005375 9.62 ADIPOQ LEP RETN TNFRSF1A
4 integument MP:0010771 9.35 ADIPOQ LEP MEFV NAMPT TNFRSF1A
5 liver/biliary system MP:0005370 9.02 ADIPOQ LEP MEFV RETN TNFRSF1A
Sources

Drugs & Therapeutics for Idiopathic Recurrent Pericarditis

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Drugs for Idiopathic Recurrent Pericarditis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antirheumatic Agents Phase 4
2 Interleukin 1 Receptor Antagonist Protein Phase 4
3 Pharmaceutical Solutions Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 AnakInRa for Treatment of Recurrent Idiopathic Pericarditis Completed NCT02219828 Phase 4 Anakinra;Placebo
2 A Double-blind, Randomized, Placebo-controlled Study to Evaluate the Efficacy and Safety of RPH-104 Treatment in Patients With Idiopathic Recurrent Pericarditis Recruiting NCT04692766 Phase 2, Phase 3 Placebo

Search NIH Clinical Center for Idiopathic Recurrent Pericarditis

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Genetic Tests for Idiopathic Recurrent Pericarditis

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Anatomical Context for Idiopathic Recurrent Pericarditis

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MalaCards organs/tissues related to Idiopathic Recurrent Pericarditis:

40
Heart, Myeloid
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Publications for Idiopathic Recurrent Pericarditis

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Articles related to Idiopathic Recurrent Pericarditis:

(show top 50) (show all 61)
# Title Authors PMID Year
1
[Pericarditis is inflammation of the pericardium, which rheumatologists should know]. 61
33216192 2021
2
Efficacy of anakinra for idiopathic and non-idiopathic pericarditis refractory or intolerant to conventional therapy. 61
32159368 2020
3
Hydroxychloroquine for colchicine-resistant glucocorticoid-dependent idiopathic recurrent pericarditis: A pilot observational prospective study. 61
32312616 2020
4
Novel Pharmacotherapies for Recurrent Pericarditis: Current Options in 2020. 61
32562029 2020
5
Onset of idiopathic recurrent pericarditis during pregnancy: successful management with colchicine and intravenous immunoglobulins. 61
32040030 2020
6
IL-1 Inhibitors in the Treatment of Monogenic Periodic Fever Syndromes: From the Past to the Future Perspectives. 61
33603750 2020
7
Canakinumab in colchicine resistant familial Mediterranean fever and other pediatric rheumatic diseases. 61
32419407 2020
8
The off-label use of anakinra in pediatric systemic autoinflammatory diseases. 61
33149772 2020
9
A novel therapy for an unusual problem: IL-1 receptor antagonist for recurrent post-transplant pericarditis. 61
31437316 2019
10
The Heart Matters: Contribution of Genetic Factors in Recurrent Pericarditis. 61
31507126 2019
11
Corticosteroids and immunosuppressive agents for idiopathic recurrent pericarditis. 61
30959210 2019
12
Anakinra in idiopathic recurrent pericarditis refractory to immunosuppressive therapy; a preliminary experience in seven patients. 61
30959216 2019
13
Management of idiopathic recurrent pericarditis during pregnancy. 61
30773267 2019
14
Anaphylactic reaction to anakinra in a child with steroid-dependent idiopathic recurrent pericarditis and successful management with canakinumab. 61
30931868 2019
15
Anakinra as rescue therapy for steroid-dependent idiopathic recurrent pericarditis in children: case report and literature review. 61
30511600 2019
16
Recurrent pericarditis: still idiopathic? The pros and cons of a well-honoured term. 61
30022399 2018
17
[Ten questions about constrictive pericarditis]. 61
29989598 2018
18
Management of idiopathic recurrent pericarditis in adults and in children: a role for IL-1 receptor antagonism. 61
29633070 2018
19
Use of Interleukin-1 Blockers in Pericardial and Cardiovascular Diseases. 61
29904899 2018
20
Cardiac disease in familial Mediterranean fever. 61
29051974 2018
21
Treating Heart Inflammation With Interleukin-1 Blockade in a Case of Erdheim-Chester Disease. 61
29910817 2018
22
Refractory idiopathic recurrent pericarditis: treatment with interleukin-1 receptor antagonist is an option! 61
28746150 2017
23
Recurrent pericarditis. 61
28185680 2017
24
Treatment of Adults with Idiopathic Recurrent Pericarditis: Novel Use of Immunotherapy. 61
28079270 2017
25
The Therapeutic Role of Interleukin-1 Inhibition in Idiopathic Recurrent Pericarditis: Current Evidence and Future Challenges. 61
28660191 2017
26
Recurrent Pericarditis. 61
27725173 2017
27
Anakinra: an emerging option for refractory idiopathic recurrent pericarditis: a systematic review of published evidence. 61
26090915 2016
28
Intravenous human immunoglobulins for refractory recurrent pericarditis: a systematic review of all published cases. 61
26090917 2016
29
Usefulness of Novel Immunotherapeutic Strategies for Idiopathic Recurrent Pericarditis. 61
26742476 2016
30
The Use of IL-1 Receptor Antagonist (Anakinra) in Idiopathic Recurrent Pericarditis: A Narrative Review. 61
26942035 2016
31
Anakinra for the management of resistant idiopathic recurrent pericarditis. Initial experience in 10 adult cases. 61
25165036 2014
32
Idiopathic recurrent pericarditis as an immune-mediated disease: current insights into pathogenesis and emerging treatment options. 61
25307995 2014
33
Acute pericarditis as the first manifestation of familial Mediterranean fever: a possible relationship with idiopathic recurrent pericarditis. 61
25088882 2014
34
New insights in the pathogenesis and therapy of idiopathic recurrent pericarditis in children. 61
23981280 2013
35
Idiopathic recurrent pericarditis treated successfully with tumour necrosis factor alpha blocking agents (anti-TNF-α). 61
23711245 2013
36
Successful treatment of idiopathic recurrent pericarditis with interleukin-1 receptor antagonist (Anakinra). 61
23463131 2013
37
Leptin, adiponectin, resistin, visfatin serum levels and idiopathic recurrent pericarditis: biomarkers of disease activity? A preliminary report. 61
23137677 2013
38
Role of autoimmunity and autoinflammation in the pathogenesis of idiopathic recurrent pericarditis. 61
21170606 2013
39
Efficacy of an interleukin-1β receptor antagonist (anakinra) in idiopathic recurrent pericarditis. 61
23052671 2013
40
Successful treatment of adult patients with idiopathic recurrent pericarditis with an interleukin-1 receptor antagonist (anakinra). 61
22704870 2012
41
Resolution of idiopathic recurrent pericarditis in a psoriatic arthritis patient treated with etanercept. 61
22155373 2012
42
A case series and systematic literature review of anakinra and immunosuppression in idiopathic recurrent pericarditis. 61
30534275 2011
43
The role of the immunogenetic background in the development and recurrence of acute idiopathic pericarditis. 61
21411999 2011
44
Recurrent pericarditis after chemotherapy for acute myeloid leukemia: a case report and a modern approach to chemotherapy-induced pericarditis. 61
22179126 2011
45
Familial clustering of recurrent pericarditis may disclose tumour necrosis factor receptor-associated periodic syndrome. 61
20497634 2010
46
Idiopathic recurrent pericarditis refractory to colchicine treatment can reveal tumor necrosis factor receptor-associated periodic syndrome. 61
20074469 2009
47
Idiopathic recurrent pericarditis: searching for Ariadne's thread. 61
19767277 2009
48
Antinuclear antibodies in recurrent idiopathic pericarditis: prevalence and clinical significance. 61
18674829 2009
49
Pathogenetic mechanisms, new drugs, and old problems in idiopathic recurrent pericarditis: comment on the article by Picco et al. 61
19644862 2009
50
Successful treatment of idiopathic recurrent pericarditis in children with interleukin-1beta receptor antagonist (anakinra): an unrecognized autoinflammatory disease? 61
19116906 2009
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Variations for Idiopathic Recurrent Pericarditis

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Expression for Idiopathic Recurrent Pericarditis

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Search GEO for disease gene expression data for Idiopathic Recurrent Pericarditis.
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Pathways for Idiopathic Recurrent Pericarditis

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Pathways related to Idiopathic Recurrent Pericarditis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Common Cytokine Receptor Gamma-Chain Family Signaling Pathways 64
Show member pathways
 12.03 TNFRSF1A RETN NAMPT LEP ADIPOQ
3
Mesenchymal Stem Cell Differentiation Pathways and Lineage-specific Markers 64
11.38 LEP ADIPOQ
4
Adipocytokine signaling pathway 36
11.17 TNFRSF1A LEP ADIPOQ
5
Adipogenesis 1
11.04 RETN NAMPT LEP ADIPOQ
6
Differentiation of white and brown adipocyte 1
10.77 LEP ADIPOQ
7
Transcription factor regulation in adipogenesis 1
10.66 LEP ADIPOQ
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GO Terms for Idiopathic Recurrent Pericarditis

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Cellular components related to Idiopathic Recurrent Pericarditis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.35 TNFRSF1A RETN NAMPT LEP ADIPOQ
2 extracellular space GO:0005615 9.02 TNFRSF1A RETN NAMPT LEP ADIPOQ

Biological processes related to Idiopathic Recurrent Pericarditis according to GeneCards Suite gene sharing:

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 cellular response to insulin stimulus GO:0032869 9.55 LEP ADIPOQ
2 signal transduction GO:0007165 9.55 TNFRSF1A RETN NAMPT LEP ADIPOQ
3 positive regulation of cold-induced thermogenesis GO:0120162 9.54 LEP ADIPOQ
4 female pregnancy GO:0007565 9.52 NAMPT LEP
5 response to nutrient GO:0007584 9.51 LEP ADIPOQ
6 response to insulin GO:0032868 9.49 RETN LEP
7 response to nutrient levels GO:0031667 9.48 LEP ADIPOQ
8 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.46 TNFRSF1A LEP
9 glucose metabolic process GO:0006006 9.43 LEP ADIPOQ
10 negative regulation of autophagy GO:0010507 9.4 NAMPT LEP
11 response to activity GO:0014823 9.37 LEP ADIPOQ
12 fatty acid beta-oxidation GO:0006635 9.32 LEP ADIPOQ
13 adipose tissue development GO:0060612 9.26 NAMPT LEP
14 negative regulation of inflammatory response GO:0050728 9.13 TNFRSF1A MEFV ADIPOQ
15 circadian rhythm GO:0007623 8.8 NAMPT LEP ADIPOQ

Molecular functions related to Idiopathic Recurrent Pericarditis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.26 TNFRSF1A NAMPT MEFV ADIPOQ
2 hormone activity GO:0005179 8.8 RETN LEP ADIPOQ
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Sources for Idiopathic Recurrent Pericarditis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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