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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 ADIPOQ (Adiponectin, C1Q And Collagen Domain Containing), and among its related pathways/superpathways are Common Cytokine Receptor Gamma-Chain Family Signaling Pathways and Circadian rythm related genes. The drugs Interleukin 1 Receptor Antagonist Protein and Antirheumatic Agents have been mentioned in the context of this disorder. Affiliated tissues include heart, bone 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 30.0 TNFRSF1A MEFV
2 periodic fever, familial, autosomal dominant 29.9 TNFRSF1A MEFV
3 hereditary periodic fever syndrome 29.8 TNFRSF1A MEFV
4 pericarditis 29.1 TNFRSF1A RETN MEFV LEP ADIPOQ
5 nontuberculous mycobacterial lung disease 10.2 LEP ADIPOQ
6 mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome 10.1 LEP ADIPOQ
7 lipodystrophy, congenital generalized, type 1 10.1 LEP ADIPOQ
8 complete generalized lipodystrophy 10.1 LEP ADIPOQ
9 adult syndrome 10.1 LEP ADIPOQ
10 brucellosis 10.1
11 lipodystrophy, familial partial, type 2 10.1 LEP ADIPOQ
12 prediabetes syndrome 10.1 LEP ADIPOQ
13 acanthosis nigricans 10.1 LEP ADIPOQ
14 inflammatory myopathy with abundant macrophages 10.0 TNFRSF1A MEFV
15 intermittent hydrarthrosis 10.0 TNFRSF1A MEFV
16 palindromic rheumatism 10.0 TNFRSF1A MEFV
17 autoimmune disease 10.0
18 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
19 constrictive pericarditis 10.0
20 cervical adenitis 10.0 TNFRSF1A MEFV
21 severe pre-eclampsia 10.0 LEP ADIPOQ
22 wells syndrome 10.0 TNFRSF1A MEFV
23 relapsing fever 10.0 TNFRSF1A MEFV
24 pellagra 10.0 NAMPT ADIPOQ
25 mevalonic aciduria 10.0 TNFRSF1A MEFV
26 sleep disorder 10.0 LEP ADIPOQ
27 adult-onset still's disease 10.0 TNFRSF1A MEFV
28 cinca syndrome 10.0 TNFRSF1A MEFV
29 carotid stenosis 9.9 NAMPT ADIPOQ
30 proteasome-associated autoinflammatory syndrome 1 9.9 MEFV ADIPOQ
31 eating disorder 9.9 LEP ADIPOQ
32 muckle-wells syndrome 9.9 TNFRSF1A MEFV
33 hypothalamic obesity 9.9 RETN LEP
34 scleroderma, familial progressive 9.9
35 sjogren syndrome 9.9
36 psoriatic arthritis 9.9
37 hemopericardium 9.9
38 cardiac tamponade 9.9
39 pericardial effusion 9.9
40 hellp syndrome 9.9
41 mixed connective tissue disease 9.9
42 connective tissue disease 9.9
43 polyarteritis nodosa 9.9
44 lipid storage disease 9.8 LEP ADIPOQ
45 intracranial hypertension 9.8 RETN ADIPOQ
46 familial hyperlipidemia 9.7 LEP ADIPOQ
47 uremia 9.6 RETN LEP
48 obesity-hypoventilation syndrome 9.6 RETN LEP ADIPOQ
49 acquired generalized lipodystrophy 9.6 RETN LEP ADIPOQ
50 fatty liver disease, nonalcoholic 1 9.6 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:

45
# 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
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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 Interleukin 1 Receptor Antagonist Protein Phase 4
2 Antirheumatic Agents Phase 4

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 AnakInRa for Treatment of Recurrent Idiopathic Pericarditis Completed NCT02219828 Phase 4 Anakinra;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, Bone, 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 58)
# Title Authors PMID Year
1
Hydroxychloroquine for colchicine-resistant glucocorticoid-dependent idiopathic recurrent pericarditis: A pilot observational prospective study. 61
32312616 2020
2
Novel Pharmacotherapies for Recurrent Pericarditis: Current Options in 2020. 61
32562029 2020
3
Onset of idiopathic recurrent pericarditis during pregnancy: successful management with colchicine and intravenous immunoglobulins. 61
32040030 2020
4
Efficacy of anakinra for idiopathic and non-idiopathic pericarditis refractory or intolerant to conventional therapy. 61
32159368 2020
5
Canakinumab in colchicine resistant familial Mediterranean fever and other pediatric rheumatic diseases. 61
32419407 2020
6
A novel therapy for an unusual problem: IL-1 receptor antagonist for recurrent post-transplant pericarditis. 61
31437316 2019
7
The Heart Matters: Contribution of Genetic Factors in Recurrent Pericarditis. 61
31507126 2019
8
Corticosteroids and immunosuppressive agents for idiopathic recurrent pericarditis. 61
30959210 2019
9
Anakinra in idiopathic recurrent pericarditis refractory to immunosuppressive therapy; a preliminary experience in seven patients. 61
30959216 2019
10
Management of idiopathic recurrent pericarditis during pregnancy. 61
30773267 2019
11
Anaphylactic reaction to anakinra in a child with steroid-dependent idiopathic recurrent pericarditis and successful management with canakinumab. 61
30931868 2019
12
Anakinra as rescue therapy for steroid-dependent idiopathic recurrent pericarditis in children: case report and literature review. 61
30511600 2019
13
Recurrent pericarditis: still idiopathic? The pros and cons of a well-honoured term. 61
30022399 2018
14
[Ten questions about constrictive pericarditis]. 61
29989598 2018
15
Management of idiopathic recurrent pericarditis in adults and in children: a role for IL-1 receptor antagonism. 61
29633070 2018
16
Use of Interleukin-1 Blockers in Pericardial and Cardiovascular Diseases. 61
29904899 2018
17
Treating Heart Inflammation With Interleukin-1 Blockade in a Case of Erdheim-Chester Disease. 61
29910817 2018
18
Cardiac disease in familial Mediterranean fever. 61
29051974 2018
19
Refractory idiopathic recurrent pericarditis: treatment with interleukin-1 receptor antagonist is an option! 61
28746150 2017
20
Recurrent pericarditis. 61
28185680 2017
21
Treatment of Adults with Idiopathic Recurrent Pericarditis: Novel Use of Immunotherapy. 61
28079270 2017
22
The Therapeutic Role of Interleukin-1 Inhibition in Idiopathic Recurrent Pericarditis: Current Evidence and Future Challenges. 61
28660191 2017
23
Recurrent Pericarditis. 61
27725173 2017
24
Anakinra: an emerging option for refractory idiopathic recurrent pericarditis: a systematic review of published evidence. 61
26090915 2016
25
Intravenous human immunoglobulins for refractory recurrent pericarditis: a systematic review of all published cases. 61
26090917 2016
26
Usefulness of Novel Immunotherapeutic Strategies for Idiopathic Recurrent Pericarditis. 61
26742476 2016
27
The Use of IL-1 Receptor Antagonist (Anakinra) in Idiopathic Recurrent Pericarditis: A Narrative Review. 61
26942035 2016
28
Anakinra for the management of resistant idiopathic recurrent pericarditis. Initial experience in 10 adult cases. 61
25165036 2014
29
Idiopathic recurrent pericarditis as an immune-mediated disease: current insights into pathogenesis and emerging treatment options. 61
25307995 2014
30
Acute pericarditis as the first manifestation of familial Mediterranean fever: a possible relationship with idiopathic recurrent pericarditis. 61
25088882 2014
31
Idiopathic recurrent pericarditis treated successfully with tumour necrosis factor alpha blocking agents (anti-TNF-α). 61
23711245 2013
32
New insights in the pathogenesis and therapy of idiopathic recurrent pericarditis in children. 61
23981280 2013
33
Successful treatment of idiopathic recurrent pericarditis with interleukin-1 receptor antagonist (Anakinra). 61
23463131 2013
34
Leptin, adiponectin, resistin, visfatin serum levels and idiopathic recurrent pericarditis: biomarkers of disease activity? A preliminary report. 61
23137677 2013
35
Role of autoimmunity and autoinflammation in the pathogenesis of idiopathic recurrent pericarditis. 61
21170606 2013
36
Efficacy of an interleukin-1β receptor antagonist (anakinra) in idiopathic recurrent pericarditis. 61
23052671 2013
37
Successful treatment of adult patients with idiopathic recurrent pericarditis with an interleukin-1 receptor antagonist (anakinra). 61
22704870 2012
38
Resolution of idiopathic recurrent pericarditis in a psoriatic arthritis patient treated with etanercept. 61
22155373 2012
39
A case series and systematic literature review of anakinra and immunosuppression in idiopathic recurrent pericarditis. 61
30534275 2011
40
Recurrent pericarditis after chemotherapy for acute myeloid leukemia: a case report and a modern approach to chemotherapy-induced pericarditis. 61
22179126 2011
41
The role of the immunogenetic background in the development and recurrence of acute idiopathic pericarditis. 61
21411999 2011
42
Familial clustering of recurrent pericarditis may disclose tumour necrosis factor receptor-associated periodic syndrome. 61
20497634 2010
43
Idiopathic recurrent pericarditis refractory to colchicine treatment can reveal tumor necrosis factor receptor-associated periodic syndrome. 61
20074469 2009
44
Idiopathic recurrent pericarditis: searching for Ariadne's thread. 61
19767277 2009
45
Antinuclear antibodies in recurrent idiopathic pericarditis: prevalence and clinical significance. 61
18674829 2009
46
Pathogenetic mechanisms, new drugs, and old problems in idiopathic recurrent pericarditis: comment on the article by Picco et al. 61
19644862 2009
47
Successful treatment of idiopathic recurrent pericarditis in children with interleukin-1beta receptor antagonist (anakinra): an unrecognized autoinflammatory disease? 61
19116906 2009
48
Recurrent pericarditis as the initial manifestation of Familial Mediterranean fever. 61
19043372 2008
49
Prognosis of idiopathic recurrent pericarditis as determined from previously published reports. 61
17826391 2007
50
[18-year-old patient with breathing-related chest pain. Idiopathic, recurrent pericarditis]. 61
14730718 2003
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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 Cells 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 16)
# Name GO ID Score Top Affiliating Genes
1 response to ethanol GO:0045471 9.56 LEP ADIPOQ
2 cellular response to insulin stimulus GO:0032869 9.55 LEP ADIPOQ
3 signal transduction GO:0007165 9.55 TNFRSF1A RETN NAMPT LEP ADIPOQ
4 positive regulation of cold-induced thermogenesis GO:0120162 9.54 LEP ADIPOQ
5 female pregnancy GO:0007565 9.52 NAMPT LEP
6 response to nutrient GO:0007584 9.51 LEP ADIPOQ
7 response to insulin GO:0032868 9.49 RETN LEP
8 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.48 TNFRSF1A LEP
9 response to nutrient levels GO:0031667 9.46 LEP ADIPOQ
10 glucose metabolic process GO:0006006 9.43 LEP ADIPOQ
11 negative regulation of autophagy GO:0010507 9.4 NAMPT LEP
12 fatty acid beta-oxidation GO:0006635 9.37 LEP ADIPOQ
13 response to activity GO:0014823 9.32 LEP ADIPOQ
14 adipose tissue development GO:0060612 9.26 NAMPT LEP
15 negative regulation of inflammatory response GO:0050728 9.13 TNFRSF1A MEFV ADIPOQ
16 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 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
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
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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