Psoriasis-related juvenile idiopathic arthritis
Synonyms: Juvenile psoriatic arthritis · Psoriasis-related JIA
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adolescent, Childhood
- Status
- active
A rare pediatric inflammatory rheumatic disease characterized by the presence of arthritis accompanied by either psoriasis or at least two of the following supporting features; presence of nail pitting, onycholysis, dactylitis, or a family history of psoriasis in a first degree relative. Patients are younger than 16 years of age and the disease lasts longer than 6 weeks.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Psoriasis-related juvenile idiopathic arthritis | Juvenile psoriatic arthritis · Psoriasis-related JIA |
| fr | Arthrite juvénile idiopathique associée au psoriasis | Arthrite psoriasique juvénile · AJI associée au psoriasis |
| de | Arthritis, idiopathische juvenile, Psoriasis-assoziierte | Juvenile Psoriasis-Arthritis · Psoriasis-assoziierte JIA |
| es | Artritis idiopática juvenil asociada a psoriasis | AIJ asociada a psoriaris · Artritis psoriásica juvenil |
| it | Artrite idiopatica giovanile correlata alla psoriasi | |
| nl | Psoriasis-gerelateerde juveniele idiopathische artritis | Juveniele psoriatische artritis · Psoriasis-gerelateerde JIA |
| pt | Artrite idiopática juvenil psoríase-relacionada | Artrite psoriática juvenil |
| pl | Młodzieńcze zapalenie stawów związane z łuszczycą | JIA związane z łuszczycą · Młodzieńcze łuszczycowe zapalenie stawów |
| cs | Juvenilní psoriatická artritida | = en |
| uk | Псоріаз-асоційований ювенільний ідіопатичний артрит | Ювенільний псоріатичний артрит · ЮРА, повязаний з псоріазом |
| zh | 银屑病相关幼年特发性关节炎 | 青少年银屑病性关节炎 |
| tr | Psöriazise bağlı jüvenil idiyopatik artrit | Sedef hastalığı-ilişkili JIA · Jüvenil psoriatik artrit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L40.5+ | NTBT | attributed | validated |
| ICD-10 | M09.0* | NTBT | attributed | validated |
| ICD-11 | FA24.2 | E | specific | validated |
| MONDO | 0019436 | E | validated | |
| UMLS | C3714758 | E | validated | |
| GARD | 10970 | E | validated | |
| MedDRA | 10076674 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:92Juvenile idiopathic arthritis
rare respiratory diseases; rare systemic and rheumatological diseases; rare systemic or rheumatologic diseases of childhood; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 24210707[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
29 annotations · validated 2019-06-06| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0011118 | Abnormality of tumor necrosis factor secretion | |
| Frequent (79-30%) | ||
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0002829 | Arthralgia | |
| HP:0001369 | Arthritis | |
| HP:0002960 | Autoimmunity | |
| HP:0100686 | Enthesitis | |
| HP:0031090 | Finger dactylitis | |
| HP:0001803 | Nail pits | |
| HP:0040313 | Oligoarthritis | |
| HP:0005764 | Polyarticular arthritis | |
| HP:0000989 | Pruritus | |
| HP:0003765 | Psoriasiform dermatitis | |
| HP:0031091 | Toe dactylitis | |
| Occasional (29-5%) | ||
| HP:0002815 | Abnormality of the knee | |
| HP:0003043 | Abnormality of the shoulder | |
| HP:0003019 | Abnormality of the wrist | |
| HP:0012122 | Anterior uveitis | |
| HP:0005197 | Generalized morning stiffness | |
| HP:0001376 | Limitation of joint mobility | |
| HP:0025300 | Malar rash | |
| HP:0025526 | Psoriasiform lesion | |
| HP:0007663 | Reduced visual acuity | |
| HP:0000988 | Skin rash | |
| HP:0000554 | Uveitis | |
| Very rare (<4-1%) | ||
| HP:0010754 | Abnormality of the temporomandibular joint | |
| HP:0001094 | Iridocyclitis | |
| HP:0001101 | Iritis | |
| HP:0001806 | Onycholysis | |
| HP:0012317 | Sacroiliac arthritis | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 29101009[PMID]_17162381[PMID]_28148698[PMID]_28222745[PMID]_28752373[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/85436
- This record as JSON: /data/d/85436.json