ORPHA:727
Microscopic polyangiitis
Synonyms: MPA · Micropolyangiitis · Microscopic polyarteritis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Childhood
- Status
- active
A rare inflammatory, necrotizing, systemic vasculitis that affects predominantly small vessels (i.e. small arteries, arterioles, capillaries, venules) in multiple organs, including the kidney, the lungs, the skin and the peripheral nerves.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Microscopic polyangiitis | MPA · Micropolyangiitis · Microscopic polyarteritis |
| fr | Polyangéite microscopique | MPA · Micropolyangéite · PAM · Polyartérite microscopique |
| de | Polyangiitis, mikroskopische | MPA · Mikropolyangiitis · Mikroskopische Panarteriitis |
| es | Poliangeitis microscópica | MPA · Micropoliangeítis · Poliangeítis microscópica · Poliarteritis microscópica |
| it | Poliangioite microscopica | MPA · Micropoliangioite · Poliarterite microscopica |
| nl | Microscopische polyangiitis | MPA · Micropolyangiitis · Microscopische polyarteriitis |
| pt | Poliangeíte microscópica | |
| pl | Mikroskopowe zapalenie naczyń | Mikroskopowe zapalenie tętnic · MPA |
| cs | Mikroskopická polyangiitida | MPA · Mikropolyangiitida · Mikroskopická polyarteritida |
| uk | Мікроскопічний поліангіїт | Мікрополіангіїт · Мікроскопічний поліартеріїт · MPA |
| zh | 显微镜下多血管炎 | 显微镜下多动脉炎 |
| tr | Mikroskobik polianjiit | MPA · Mikropolianjit · Mikroskobik poliarterit |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M31.7 | E | specific | validated |
| ICD-11 | 4A44.A0 | E | specific | validated |
| MONDO | 0019124 | E | validated | |
| MeSH | D055953 | E | validated | |
| UMLS | C2347126 | E | validated | |
| GARD | 3652 | E | validated | |
| MedDRA | 10063344 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:156152Anti-neutrophil cytoplasmic antibody-associated vasculitis
rare respiratory diseases; rare systemic and rheumatological diseases; rare circulatory system diseases; rare systemic or rheumatologic diseases of childhood; rare transplant-related disorders - ORPHA:209007Systemic inflammatory disease associated with an acquired peripheral neuropathy
rare neurological diseases - ORPHA:567560Systemic vasculitis associated with glomerulopathy
rare renal diseases; rare transplant-related disorders
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
21 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 1 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| Annual incidence | 1-9 / 1 000 000 | 0.26 / 100 000 | Germany | Value and class | Validated | 15696553[PMID]_25805746[PMID] |
| Point prevalence | 1-9 / 100 000 | 4.2843 / 100 000 | Worldwide | Value and class | Validated | 25805746[PMID]_ORPHANET |
| Annual incidence | 1-9 / 100 000 | 1.16 / 100 000 | Spain | Value and class | Validated | 11156552[PMID] |
| Point prevalence | 1-9 / 100 000 | 9.4 / 100 000 | Sweden | Value and class | Validated | 17553910[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.5 / 100 000 | Australia | Value and class | Validated | 18771432[PMID] |
| Point prevalence | 1-9 / 100 000 | 2.5 / 100 000 | France | Value and class | Validated | 14872461[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.27 / 100 000 | Norway | Value and class | Validated | 11760724[PMID] |
| Point prevalence | 1-9 / 100 000 | 3.7 / 100 000 | New Zealand | Value and class | Validated | 16368729[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.74 / 100 000 | United Kingdom | Value and class | Not yet validated | 11156552[PMID]_22258386[PMID]_10693883[PMID]_ORPHANET |
| Point prevalence | 1-9 / 100 000 | 6.3 / 100 000 | United Kingdom | Value and class | Validated | 22258386[PMID]_25805746[PMID] |
| Point prevalence | 1-9 / 100 000 | 2.8 / 100 000 | Germany | Value and class | Validated | 24425780[PMID]_25805746[PMID] |
| Point prevalence | 1-9 / 100 000 | 3.91 / 100 000 | Australia | Value and class | Validated | 18771432[PMID]_25805746[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.38 / 100 000 | Japan | Value and class | Validated | 17699321[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 100 000 | 2.4 / 100 000 | Kuwait | Value and class | Validated | 9306338[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.82 / 100 000 | Japan | Value and class | Validated | 21798892[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.71 / 100 000 | Canada | Value and class | Validated | 23838025[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.02 / 100 000 | Greece | Value and class | Validated | 19604432[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.3 / 100 000 | Lithuania | Value and class | Validated | 15647446[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.01 / 100 000 | Sweden | Value and class | Validated | 19797309[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.6 / 100 000 | United States | Value and class | Validated | 28881446[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.
6
HPO phenotypes
55 annotations · validated 2024-10-03| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002960 | Autoimmunity | |
| HP:0008653 | Crescentic glomerulonephritis | |
| HP:0010783 | Erythema | |
| HP:0001945 | Fever | |
| HP:0000099 | Glomerulonephritis | |
| HP:0100820 | Glomerulopathy | |
| HP:0000790 | Hematuria | |
| HP:0002105 | Hemoptysis | |
| HP:0012649 | Increased inflammatory response | |
| HP:0100520 | Oliguria | |
| HP:0000083 | Renal insufficiency | |
| HP:0000988 | Skin rash | |
| HP:0002633 | Vasculitis | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0012614 | Abnormal urine cytology | |
| HP:0001903 | Anemia | |
| HP:0033559 | Anti-myeloperoxidase antibody positivity | |
| HP:0034104 | Anti-neutrophil elastase antibody positivity | |
| HP:0033557 | Anti-proteinase 3 antibody positivity | |
| HP:0002829 | Arthralgia | |
| HP:0032230 | Cytoplasmic antineutrophil antibody positivity | |
| HP:0002014 | Diarrhea | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0012378 | Fatigue | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0005244 | Gastrointestinal infarctions | |
| HP:0033505 | Livedo reticularis | |
| HP:0032018 | Multiple mononeuropathy | |
| HP:0003326 | Myalgia | |
| HP:0002017 | Nausea and vomiting | |
| HP:0002586 | Peritonitis | |
| HP:0004396 | Poor appetite | |
| HP:0200042 | Skin ulcer | |
| HP:0001933 | Subcutaneous hemorrhage | |
| HP:0004936 | Venous thrombosis | |
| Occasional (29-5%) | ||
| HP:0008046 | Abnormal retinal vascular morphology | |
| HP:0011675 | Arrhythmia | |
| HP:0001369 | Arthritis | |
| HP:0100749 | Chest pain | |
| HP:0001635 | Congestive heart failure | |
| HP:0000965 | Cutis marmorata | |
| HP:0025420 | Diffuse alveolar hemorrhage | |
| HP:0100534 | Episcleritis | |
| HP:0000421 | Epistaxis | |
| HP:0100758 | Gangrene | |
| HP:0031363 | Palpable purpura | |
| HP:0001733 | Pancreatitis | |
| HP:0003401 | Paresthesia | |
| HP:0001701 | Pericarditis | |
| HP:0009830 | Peripheral neuropathy | |
| HP:0025188 | Retinal vasculitis | |
| HP:0000246 | Sinusitis | |
| HP:0001482 | Subcutaneous nodule | |
| HP:0000554 | Uveitis | |
| HP:0001824 | Weight loss | |
Run the differential on the 12 obligate / very frequent terms
Annotation source: 30285379[PMID]
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/727
- This record as JSON: /data/d/727.json