47:
90:
644:
231:
530:
592:. These granulomas are the main reason for the name granulomatosis with polyangiitis, although it is not an essential feature. Nevertheless, necrotizing granulomas are a hallmark of this disease. However, many biopsies can be nonspecific and 50% provide too little information for the diagnosis of GPA.
140:
and a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys. The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms
871:
In generalized non-organ-threatening disease, remission can be achieved with a combination of methotrexate and corticosteroids, where the steroid dose is reduced after a remission has been achieved and methotrexate is used as maintenance therapy. Treatment measures for localised GPA of the nose and
834:
Rituximab may be substituted for cyclophosphamide to induce remission since it is similarly effective and has a comparable side effect profile. The dose of corticosteroids is generally tapered (decreased) very slowly over the course of several months to reduce the risk of another GPA flare. After a
809:
Oral and intravenous cyclophosphamide are both effective for induction of GPA remission. Oral cyclophosphamide at a dose of 2 mg/kg/day was the standard treatment for many years; this regimen resulted in complete remission in more than 75% of people with GPA but is associated with significant
710:
898:. The revised Five-factor score is associated with 5-year mortality from GPA and is based on the following criteria: age greater than 65 years, cardiac symptoms, gastrointestinal involvement, chronic kidney disease, and the absence of ears, nose, and throat symptoms.
2536:
Falk RJ, Gross WL, Guillevin L, Hoffman G, Jayne DR, Jennette JC, Kallenberg CG, Luqmani R, Mahr AD, Matteson EL, Merkel PA, Specks U, Watts R (April 2011). "Granulomatosis with polyangiitis (Wegener's): an alternative name for
Wegener's granulomatosis".
826:
by one-third. However, pulsed intravenous cyclophosphamide may be associated with a higher risk of GPA relapse when compared to oral cyclophosphamide. Due to a high frequency of abnormally low white blood cell counts seen with cyclophosphamide treatment,
541:
can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. More than 90% of people who have GPA test positive for ANCA. Cytoplasmic-staining ANCAs that react with the enzyme
1946:
Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW (August 1990). "The
American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis".
835:
person with GPA has successfully undergone induction and gone into remission, the treatment goal then shifts to maintenance of remission and preventing subsequent GPA flares. Less toxic immunosuppressing medications such as
1982:
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG (February 1994). "Nomenclature of systemic vasculitides. Proposal of an international consensus conference".
916:
Today, medication toxicity is managed more carefully and long-term remissions are possible. Some affected individuals are able to lead relatively normal lives and remain in remission for 20+ years after treatment.
2163:
Stone, John H.; Merkel, Peter A.; Spiera, Robert; Seo, Philip; Langford, Carol A.; Hoffman, Gary S.; Kallenberg, Cees G.M.; St. Clair, E. William; Turkiewicz, Anthony; Tchao, Nadia K.; Webber, Lisa (2010-07-15).
2629:
2614:
2599:
1529:
Pakalniskis, MG; Berg, AD; Policeni, BA; Gentry, LR; Sato, Y; Moritani, T; Smoker, WR (December 2015). "The Many Faces of
Granulomatosis With Polyangiitis: A Review of the Head and Neck Imaging Manifestations".
2059:
Robson, Joanna C.; Grayson, Peter C.; Ponte, Cristina; Suppiah, Ravi; Craven, Anthea; Judge, Andrew; Khalid, Sara; Hutchings, Andrew; Watts, Richard A.; Merkel, Peter A.; Luqmani, Raashid A. (2022-03-01).
872:
sinuses includes nasal irrigation, nasal corticosteroids, and antibiotics if infection occurs. If perforation of the nasal septum occurs (or saddle nose deformity), then surgical repair is recommended.
2221:
Tracy, CL; Papadopoulos, PJ; Bye, MR; Connolly, H; Goldberg, E; O'Brian, RJ; Sharma, GD; Talavera, F; Toder, DS; Valentini, RP; Windle, ML; Wolf, RE (10 February 2014). Diamond, HS (ed.).
1854:
Tracy, CL; Papadopoulos, PJ; Bye, MR; Connolly, H; Goldberg, E; O'Brian, RJ; Sharma, GD; Talavera, F; Toder, DS; Valentini, RP; Windle, ML; Wolf, RE (10 February 2014). Diamond, HS (ed.).
1357:
Millet, A; Pederzoli-Ribeil, M; Guillevin, L; Witko-Sarsat, V; Mouthon, L (August 2013). "Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group?".
822:. In contrast, administering pulsed doses of intravenous cyclophosphamide is equally effective for inducing remission, results in a lower cumulative dose, and decreases the incidence of
2299:
1920:
554:) are associated with GPA. Involvement of the ears, nose, and throat is more common in granulomatosis with polyangiitis than in the similar condition microscopic polyangiitis.
913:. The risk of relapse is increased in people with GPA who test positive for anti-PR3 ANCA antibodies and is higher than the relapse risk for microscopic polyangiitis.
3282:
1221:
Lally, L; Spiera, R (2015). "Current landscape of antineutrophil cytoplasmic antibody-associated vasculitis: classification, diagnosis, and treatment".
1124:
Lopalco, G; Rigante, D; Venerito, V; Emmi, G; Anelli, MG; Lapadula, G; Iannone, F; Cantarini, L (June 2016). "Management of Small Vessel
Vasculitides".
2062:"2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis"
1655:
Berden A, Göçeroglu A, Jayne D, Luqmani R, Rasmussen N, Bruijn JA, Bajema I (January 2012). "Diagnosis and management of ANCA associated vasculitis".
620:. Although GPA affects small- and medium-size vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system.
951:
article entitled "Photographs of a case of rapid destruction of the nose and face". Heinz Karl Ernst
Klinger (born 1907) added information on the
754:
In 2022, American
College of Rheumatology and the European Alliance of Associations for Rheumatology updated the classification criteria for GPA.
537:
Granulomatosis with polyangiitis is usually suspected only when a person has had unexplained symptoms for a long period of time. Determination of
288:
are the most common signs of GPA in the eye; involvement of the eyes is common and occurs in slightly more than half of people with the disease.
3292:
3067:
1053:
258:, is seen in nearly all people with GPA. Typical signs and symptoms of nose or sinus involvement include crusting around the nose, stuffiness,
3287:
3206:
2879:
2773:
613:
20:
2998:
3184:
3145:
3062:
1565:
Treitman, P; Herskowitz, JL; Bass, HN (August 1991). "Churg-Strauss syndrome in a 12-year-old boy diagnosed by transbronchial biopsy".
3123:
2307:
1078:
Singer, O; McCune, WJ (May 2017). "Update on maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis".
3072:
2712:
2493:
Mendenhall WM, Olivier KR, Lynch JW, Mendenhall NP (April 2006). "Lethal midline granuloma-nasal natural killer/T-cell lymphoma".
222:
3 cases per 100,000 people in the United States and equally affects men and women. GPA has infrequently been reported in minors.
3157:
3114:
296:
2415:
Friedmann I (January 1982). "McBride and the midfacial granuloma syndrome. (The second 'McBride
Lecture', Edinburgh, 1980)".
1259:
Keller, SF; Miloslavsky, EM (February 2016). "Corticosteroids in
Antineutrophil Cytoplasmic Antibody-Associated Vasculitis".
2706:
538:
486:
890:
Before modern treatments, the 2-year survival was under 10% and average survival five months. Death usually resulted from
462:
has been hypothesized as an initiating factor of the autoimmunity seen in people with GPA. Several genes involved in the
3039:
2925:
1037:
2940:
2904:
2766:
2991:
2728:
1029:
828:
729:
629:
1827:
Marzano, AV; Balice, Y; Tavecchio, S; Desimine, C; Colombo, A; Berti, E (April 2015). "Granulomatous vasculitis".
334:, underlying bone destruction with loosening of teeth, non-specific ulcerations throughout the lining of the mouth
3167:
2935:
2909:
171:
GPA treatment depends on the severity of the disease. Severe disease is typically treated with a combination of
1033:
632:
accepted classification criteria for GPA. These criteria were not intended for diagnosis, but for inclusion in
1040:(EULAR) resolved to change the name to granulomatosis with polyangiitis, given Wegener's association with the
242:
Initial signs are highly variable, and diagnosis can be severely delayed due to the nonspecific nature of the
3044:
875:
251:
604:
or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating
3262:
3257:
2759:
533:
Immunofluorescence pattern produced by binding of ANCA to ethanol-fixed neutrophils, from a person with GPA
2717:
2984:
2930:
964:
787:
637:
633:
3026:
2739:
489:(ANCAs) are responsible for the inflammation in GPA. The typical ANCAs in GPA are those that react with
444:
Classic microscopic features of GPA include inflammation of blood vessels associated with poorly formed
3093:
2831:
3149:
3054:
2896:
2086:
732:(CHCC) on the nomenclature of systemic vasculitis (1992), establishing the diagnosis of GPA demands:
219:
1707:
Kuan, EC; Suh, JD (February 2017). "Systemic and
Odontogenic Etiologies in Chronic Rhinosinusitis".
210:
of GPA each year is estimated to be 2.1–14.4 new cases per million people in Europe. GPA is rare in
89:
3201:
3127:
2884:
2644:
2120:
Schönermarck, U; Gross, WL; de Groot, K (January 2014). "Treatment of ANCA-associated vasculitis".
960:
926:
803:
795:
617:
207:
2372:
Cartin-Ceba R, Peikert T, Specks U (December 2012). "Pathogenesis of ANCA-associated vasculitis".
3267:
2798:
2222:
1912:
948:
479:
2813:
1855:
1611:
Seo P, Stone JH (July 2004). "The antineutrophil cytoplasmic antibody-associated vasculitides".
3297:
3034:
2841:
2790:
906:
547:
517:. In theory, this phenomenon could cause extensive damage to the vessel wall, in particular of
494:
408:
399:
300:
2684:
2608:
218:
populations but occurs more often in people of
Northern European descent. GPA is estimated to
46:
3277:
2965:
2330:"Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review"
852:
770:
The standard treatment for severe GPA is to induce remission with immunosuppressants such as
689:
458:
3135:
3088:
2960:
2821:
2803:
952:
815:
558:
384:
370:
356:
188:
600:
Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called
8:
3272:
2782:
2633:
1469:
Walters, Giles D.; Willis, Narelle S.; Cooper, Tess E.; Craig, Jonathan C. (2020-01-13).
895:
799:
601:
1297:
Papaliodis, GN (November 2017). "Ophthalmologic manifestations of systemic vasculitis".
3252:
3231:
3226:
3109:
3017:
2562:
2518:
2506:
2440:
2397:
2276:
2249:
2198:
2165:
2145:
1804:
1777:
1680:
1503:
1470:
1382:
1322:
1198:
1173:
1149:
1103:
791:
395:
351:: pulmonary nodules (referred to as "coin lesions"), infiltrates (often interpreted as
339:
137:
101:
2638:
1439:
1422:
2655:
2554:
2510:
2475:
2471:
2444:
2432:
2389:
2351:
2281:
2203:
2185:
2137:
2099:
2091:
2041:
2000:
1964:
1901:
1836:
1809:
1751:
1724:
1684:
1672:
1628:
1582:
1547:
1508:
1490:
1444:
1386:
1374:
1314:
1276:
1238:
1203:
1141:
1095:
975:
930:
305:
273:
172:
125:
74:
2566:
2522:
2401:
2077:
2036:
2019:
1370:
1326:
1153:
1107:
2976:
2826:
2695:
2546:
2502:
2467:
2424:
2381:
2341:
2271:
2261:
2193:
2177:
2149:
2129:
2081:
2073:
2031:
1992:
1956:
1893:
1799:
1789:
1716:
1664:
1620:
1574:
1539:
1498:
1486:
1482:
1434:
1366:
1306:
1268:
1230:
1193:
1185:
1133:
1087:
999:
902:
775:
643:
608:
termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size
589:
551:
514:
215:
180:
647:
The left apical region is opacified in a case of granulomatosis with polyangiitis.
3174:
3162:
2649:
1624:
1310:
1189:
1091:
941:
879:
200:
1743:
905:, 5-year survival is over 80%. Long-term complications are common (86%), mainly
3007:
2660:
1794:
1578:
944:
831:
is a common complication and prophylaxis against this pathogen is recommended.
819:
783:
779:
570:
391:
285:
203:
is also used in severe cases with damage to the lungs, kidneys, or intestines.
184:
2623:
2428:
2385:
1897:
1720:
1272:
1234:
1137:
762:
GPA treatment depends on its severity and whether it has caused organ damage.
3246:
2189:
2095:
1494:
882:
did not confirm fewer relapses with trimethoprim/sulfamethoxazole treatment.
856:
737:
698:
510:
463:
324:
2550:
2133:
929:
is 10–20 cases per million per year. It is exceedingly rare in Japan and in
3188:
2850:
2558:
2514:
2479:
2393:
2355:
2346:
2329:
2285:
2220:
2207:
2141:
2103:
1996:
1960:
1905:
1853:
1840:
1813:
1755:
1728:
1676:
1632:
1551:
1512:
1448:
1378:
1356:
1318:
1280:
1242:
1207:
1145:
1099:
844:
840:
609:
585:
566:
543:
490:
433:
320:
281:
196:
192:
83:
66:
2436:
2300:"Vasculitis Foundation " Granulomatosis with Polyangiitis (GPA/Wegener's)"
2181:
2045:
2020:"A comparison of two nomenclature systems for primary systemic vasculitis"
2004:
1968:
1884:
Chen, JH; Deshpande, V (June 2017). "IgG4-related Disease and the Liver".
1586:
2679:
1543:
982:
848:
811:
506:
502:
412:
404:
268:
235:
57:
showing features characteristic of granulomatosis with polyangiitis – a
2591:
786:
is sometimes recommended for very severe manifestations of GPA, such as
2701:
2061:
1041:
860:
823:
744:
453:
375:
360:
331:
263:
133:
128:, is a rare long-term systemic disorder that involves the formation of
79:
58:
54:
2751:
751:
Several investigators have compared the ACR and Chapel Hill criteria.
2690:
968:
947:
Peter McBride (1854–1946) first described the condition in 1897 in a
836:
771:
582:
518:
445:
366:
352:
309:
277:
259:
176:
168:
have a role in GPA though the risk of inheritance appears to be low.
142:
129:
62:
1668:
863:, appear to be ineffective and are not recommended for routine use.
2734:
2266:
910:
661:
605:
578:
449:
425:
343:
247:
165:
2166:"Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis"
709:
985:, in two reports in 1936 and 1939, leading to the eponymous name
979:
379:
255:
243:
230:
146:
2492:
1778:"Scleritis as the harbinger of Granulomatosis with polyangiitis"
1775:
2873:
2859:
2618:
2603:
891:
562:
468:
292:
158:
2327:
1826:
1020:
2223:"Granulomatosis with Polyangiitis Treatment & Management"
1528:
474:
429:
211:
150:
2328:
Bosch X, Guilabert A, Espinosa G, Mirapeix E (August 2007).
2723:
1654:
1014:
1008:
348:
327:
dysfunction, sensorineural hearing loss (unclear mechanism)
154:
1123:
2535:
2119:
2058:
1981:
1011:
529:
313:
16:
Autoimmune disease with chronic blood vessel inflammation
2458:
Fienberg R (December 1955). "Pathergic granulomatosis".
2371:
1776:
Kharel Sitaula R, Maskey HM, Chaudhary S, Jha S (2022).
1468:
878:
has been proposed to help prevent relapse though a 2015
424:
The cause of GPA is unknown, although microbes, such as
1945:
369:: Pain or swelling (60%), often initially diagnosed as
1564:
145:, stuffy nose and crustiness of nasal secretions, and
2250:"Progress in treatment of ANCA-associated vasculitis"
1017:
713:
Photo showing the sclerokeratitis associated with GPA
250:
is the first sign in most people. Involvement of the
3006:
2581:
2162:
1921:"Granulomatosis with Polyangiitis (GPA): Vasculitis"
1741:
1005:
588:(clumps of typically arranged white blood cells) on
2115:
2113:
1750:. Treasure Island, Florida: StatPearls Publishing.
1002:
798:). The use of plasmapheresis in those with GPA and
1524:
1522:
1423:"Current therapies for ANCA-associated vasculitis"
432:, as well as genetics have been implicated in its
378:: subcutaneous nodules (granulomas) on the elbow,
199:, or rituximab to keep the disease under control.
1258:
974:The full clinical picture was first presented by
740:inflammation involving the respiratory tract, and
565:is obtained from the kidneys. On rare occasions,
557:If the person has signs of kidney involvement or
3244:
2110:
1829:Giornale Italiano di Dermatologia e Venereologia
1292:
1290:
482:genes may influence the risk of developing GPA.
2247:
1519:
959:. The disease is still sometimes confused with
802:(renal vasculitis) might reduce progression to
638:sensitivity of 88.2% and a specificity of 92.0%
276:. Inflammation of the outer layers of the eye (
238:damage due to granulomatosis with polyangiitis.
1471:"Interventions for renal vasculitis in adults"
1054:List of medical eponyms with Nazi associations
539:anti-neutrophil cytoplasmic antibodies (ANCAs)
3207:Eosinophilic granulomatosis with polyangiitis
2992:
2880:Eosinophilic granulomatosis with polyangiitis
2767:
1883:
1742:Schonberg S, Stokkermans TJ (7 August 2023).
1287:
1214:
1119:
1117:
614:eosinophilic granulomatosis with polyangiitis
21:eosinophilic granulomatosis with polyangiitis
1558:
1416:
1352:
1350:
1348:
1346:
1344:
1342:
1340:
1338:
1336:
1254:
1252:
1077:
1073:
1071:
1069:
2408:
2248:Smith RM, Jones RB, Jayne DR (April 2012).
1879:
1877:
1820:
1702:
1700:
1698:
1696:
1694:
1475:The Cochrane Database of Systematic Reviews
1420:
1414:
1412:
1410:
1408:
1406:
1404:
1402:
1400:
1398:
1396:
1220:
1167:
1165:
1163:
501:studies have found that ANCAs can activate
3283:Steroid-responsive inflammatory conditions
2999:
2985:
2774:
2760:
2486:
1296:
1261:Rheumatic Disease Clinics of North America
1223:Rheumatic Disease Clinics of North America
1171:
1114:
816:inflammation and bleeding from the bladder
147:inflammation of the uveal layer of the eye
88:
45:
2414:
2345:
2275:
2265:
2197:
2087:1983/ba2d3ba8-c9a1-4606-a765-459e1eb54e36
2085:
2035:
2017:
1886:Gastroenterology Clinics of North America
1803:
1793:
1735:
1502:
1438:
1333:
1249:
1197:
1066:
612:. Apart from GPA, this category includes
2457:
1918:
1874:
1709:Otolaryngologic Clinics of North America
1691:
1610:
1393:
1160:
708:
642:
528:
229:
40:Wegener's granulomatosis (WG) (formerly)
3073:Membranoproliferative/mesangiocapillary
2781:
2367:
2365:
1975:
1939:
1706:
636:. Two or more positive criteria have a
248:irritation and inflammation of the nose
3245:
2417:The Journal of Laryngology and Otology
1650:
1648:
1646:
1644:
1642:
1606:
1604:
1602:
1600:
1598:
1596:
1532:AJR. American Journal of Roentgenology
824:abnormally low white blood cell counts
487:anti-neutrophil cytoplasmic antibodies
297:rapidly progressive glomerulonephritis
3293:Vascular-related cutaneous conditions
2980:
2755:
2529:
2495:American Journal of Clinical Oncology
2321:
2011:
225:
3288:Systemic connective tissue disorders
2362:
2214:
1464:
1462:
1460:
1458:
1172:Yates, M; Watts, R (February 2017).
955:. An early name for the disease was
2241:
1847:
1639:
1593:
705:Biopsy: granulomatous inflammation
485:It is now widely presumed that the
13:
2926:Acute hemorrhagic edema of infancy
2507:10.1097/01.coc.0000198738.61238.eb
1856:"Granulomatosis with Polyangiitis"
1038:European League Against Rheumatism
866:
669:Lungs: abnormal chest X-ray with:
439:
65:with multi-nucleated giant cells.
14:
3309:
2941:Cutaneous small-vessel vasculitis
2905:Cutaneous small-vessel vasculitis
2577:
1455:
1440:10.1146/annurev-med-011514-023051
765:
595:
3197:Granulomatosis with polyangiitis
3008:Disease of the kidney glomerules
2866:Granulomatosis with polyangiitis
2729:granulomatosis-with-polyangiitis
2718:granulomatosis-with-polyangiitis
2707:Granulomatosis with polyangiitis
2539:Annals of the Rheumatic Diseases
2460:The American Journal of Medicine
2254:Arthritis Research & Therapy
2066:Annals of the Rheumatic Diseases
2018:Bruce IN, Bell AL (April 1997).
1782:Annals of Medicine & Surgery
1613:The American Journal of Medicine
1359:Annals of the Rheumatic Diseases
1299:Current Opinion in Ophthalmology
1030:American College of Rheumatology
998:
829:Pneumocystis jirovecii pneumonia
747:of small to medium-size vessels.
730:Chapel Hill Consensus Conference
686:Kidneys: urinary sediment with:
655:painful or painless oral ulcers
630:American College of Rheumatology
573:examination, a biopsy will show
415:, other organs: rarely affected.
363:, and rarely bronchial stenosis.
110:Granulomatosis with polyangiitis
32:Granulomatosis with polyangiitis
2936:Bullous small vessel vasculitis
2451:
2292:
2170:New England Journal of Medicine
2156:
2078:10.1136/annrheumdis-2021-221795
2052:
2024:British Journal of Rheumatology
1769:
1371:10.1136/annrheumdis-2013-203255
1080:Current Opinion in Rheumatology
920:
1927:. Merck Sharp & Dohme Corp
1487:10.1002/14651858.CD003232.pub4
1034:American Society of Nephrology
778:in combination with high-dose
505:, increase their adherence to
456:. Bacterial colonization with
274:hole in the septum of the nose
124:), after the German physician
1:
2037:10.1093/rheumatology/36.4.453
1059:
876:Trimethoprim/sulfamethoxazole
173:immunosuppressive medications
164:The cause of GPA is unknown.
134:inflammation of blood vessels
2472:10.1016/0002-9343(55)90150-9
2374:Current Rheumatology Reports
2304:www.vasculitisfoundation.org
1625:10.1016/j.amjmed.2004.02.030
1421:Lally, L; Spiera, R (2015).
1311:10.1097/ICU.0000000000000422
1190:10.7861/clinmedicine.17-1-60
1174:"ANCA-associated vasculitis"
1126:Current Rheumatology Reports
1092:10.1097/BOR.0000000000000382
885:
757:
652:Nasal or oral inflammation:
634:randomized controlled trials
569:lung biopsy is required. On
524:
7:
3068:Endocapillary proliferative
2931:Cryoglobulinemic vasculitis
1047:
965:lymphomatoid granulomatosis
788:diffuse alveolar hemorrhage
623:
575:leukocytoclastic vasculitis
10:
3314:
2832:Thromboangiitis obliterans
2122:Nature Reviews. Nephrology
1795:10.1016/j.amsu.2022.104908
1579:10.1177/000992289103000809
936:
18:
3219:
3183:
3150:Type III hypersensitivity
3144:
3122:
3106:
3081:
3053:
3025:
3014:
2953:
2918:
2897:Type III hypersensitivity
2895:
2849:
2840:
2812:
2789:
2670:
2585:
2429:10.1017/s0022215100092197
2386:10.1007/s11926-012-0286-y
1925:Merck Manual Professional
1898:10.1016/j.gtc.2017.01.001
1721:10.1016/j.otc.2016.08.008
1427:Annual Review of Medicine
1273:10.1016/j.rdc.2015.08.010
1235:10.1016/j.rdc.2014.09.003
1138:10.1007/s11926-016-0580-1
901:With corticosteroids and
716:within the arterial wall
664:or bloody nasal discharge
493:, an enzyme prevalent in
419:
97:
73:
53:
44:
36:
31:
3202:Microscopic polyangiitis
3128:Type II hypersensitivity
2885:Microscopic polyangiitis
1985:Arthritis and Rheumatism
1949:Arthritis and Rheumatism
987:Wegener's granulomatosis
961:lethal midline granuloma
957:pathergic granulomatosis
804:end-stage kidney disease
796:pulmonary-renal syndrome
790:and rapidly progressive
722:in the perivascular area
618:microscopic polyangiitis
330:Oral cavity: strawberry
118:Wegener's granulomatosis
19:Not to be confused with
3063:Mesangial proliferative
2551:10.1136/ard.2011.150714
2134:10.1038/nrneph.2013.225
495:neutrophil granulocytes
480:human leukocyte antigen
254:, such as the nose and
252:upper respiratory tract
189:symptoms of the disease
136:(vasculitis). It is an
116:), previously known as
2347:10.1001/jama.298.6.655
1997:10.1002/art.1780370206
1961:10.1002/art.1780330807
991:Wegener granulomatosis
907:chronic kidney failure
714:
648:
534:
409:gastrointestinal tract
400:mononeuritis multiplex
382:, various others (see
301:chronic kidney disease
239:
3168:diffuse proliferative
2182:10.1056/NEJMoa0909905
1919:Gota, CE (May 2013).
853:mycophenolate mofetil
810:toxicities including
712:
690:microscopic hematuria
646:
602:systemic vasculitides
532:
459:Staphylococcus aureus
357:bleeding in the lungs
355:), cavitary lesions,
233:
3136:Goodpasture syndrome
2961:Goodpasture syndrome
2822:Polyarteritis nodosa
2804:Giant cell arteritis
2799:Takayasu's arteritis
1544:10.2214/AJR.14.13864
953:anatomical pathology
909:, hearing loss, and
800:acute kidney failure
559:cutaneous vasculitis
385:cutaneous vasculitis
371:rheumatoid arthritis
359:causing a person to
3263:Disorders of fascia
3258:Autoimmune diseases
2783:Systemic vasculitis
1567:Clinical Pediatrics
896:respiratory failure
640:of describing GPA.
509:, and induce their
272:deformity due to a
208:number of new cases
3232:glomerulonephrosis
3227:glomerulonephritis
3158:Post-streptococcal
2966:Sneddon's syndrome
2671:External resources
2227:Medscape Reference
1860:Medscape Reference
1816:. Art. No. 104908.
792:glomerulonephritis
715:
649:
535:
396:sensory neuropathy
299:(75%), leading to
240:
226:Signs and symptoms
138:autoimmune disease
102:Autoimmune disease
3240:
3239:
3215:
3214:
3102:
3101:
3027:Non-proliferative
2974:
2973:
2949:
2948:
2749:
2748:
1178:Clinical Medicine
976:Friedrich Wegener
967:, both malignant
931:African Americans
806:at three months.
728:According to the
571:histopathological
515:endothelial cells
398:(10%) and rarely
319:Ears: conductive
149:. Damage to the
126:Friedrich Wegener
107:
106:
26:Medical condition
3305:
3120:
3119:
3023:
3022:
3001:
2994:
2987:
2978:
2977:
2847:
2846:
2827:Kawasaki disease
2776:
2769:
2762:
2753:
2752:
2583:
2582:
2571:
2570:
2533:
2527:
2526:
2490:
2484:
2483:
2455:
2449:
2448:
2412:
2406:
2405:
2369:
2360:
2359:
2349:
2325:
2319:
2318:
2316:
2315:
2306:. Archived from
2296:
2290:
2289:
2279:
2269:
2245:
2239:
2238:
2236:
2234:
2218:
2212:
2211:
2201:
2160:
2154:
2153:
2117:
2108:
2107:
2089:
2056:
2050:
2049:
2039:
2015:
2009:
2008:
1979:
1973:
1972:
1943:
1937:
1936:
1934:
1932:
1916:
1910:
1909:
1881:
1872:
1871:
1869:
1867:
1851:
1845:
1844:
1824:
1818:
1817:
1807:
1797:
1773:
1767:
1766:
1764:
1762:
1739:
1733:
1732:
1704:
1689:
1688:
1652:
1637:
1636:
1608:
1591:
1590:
1562:
1556:
1555:
1526:
1517:
1516:
1506:
1466:
1453:
1452:
1442:
1418:
1391:
1390:
1354:
1331:
1330:
1294:
1285:
1284:
1256:
1247:
1246:
1218:
1212:
1211:
1201:
1169:
1158:
1157:
1121:
1112:
1111:
1075:
1028:). In 2011, the
1027:
1026:
1023:
1022:
1019:
1016:
1013:
1010:
1007:
1004:
997:
945:otolaryngologist
903:cyclophosphamide
776:cyclophosphamide
552:white blood cell
513:that can damage
216:African-American
181:cyclophosphamide
93:
92:
49:
29:
28:
3313:
3312:
3308:
3307:
3306:
3304:
3303:
3302:
3243:
3242:
3241:
3236:
3211:
3179:
3140:
3113:
3108:
3098:
3077:
3049:
3040:Focal segmental
3016:
3010:
3005:
2975:
2970:
2945:
2914:
2891:
2836:
2808:
2785:
2780:
2750:
2745:
2744:
2666:
2665:
2594:
2580:
2575:
2574:
2534:
2530:
2491:
2487:
2456:
2452:
2413:
2409:
2370:
2363:
2326:
2322:
2313:
2311:
2298:
2297:
2293:
2246:
2242:
2232:
2230:
2219:
2215:
2161:
2157:
2118:
2111:
2057:
2053:
2016:
2012:
1980:
1976:
1944:
1940:
1930:
1928:
1917:
1913:
1882:
1875:
1865:
1863:
1852:
1848:
1825:
1821:
1774:
1770:
1760:
1758:
1740:
1736:
1705:
1692:
1669:10.1136/bmj.e26
1653:
1640:
1609:
1594:
1569:(Case Report).
1563:
1559:
1527:
1520:
1481:(1): CD003232.
1467:
1456:
1419:
1394:
1355:
1334:
1295:
1288:
1257:
1250:
1219:
1215:
1170:
1161:
1122:
1115:
1076:
1067:
1062:
1050:
1001:
995:
994:
978:(1907–1990), a
939:
923:
888:
880:Cochrane review
869:
867:Limited disease
780:corticosteroids
768:
760:
626:
598:
527:
442:
440:Pathophysiology
422:
394:: occasionally
228:
201:Plasma exchange
187:to control the
185:corticosteroids
87:
27:
24:
17:
12:
11:
5:
3311:
3301:
3300:
3295:
3290:
3285:
3280:
3275:
3270:
3268:Lung disorders
3265:
3260:
3255:
3238:
3237:
3235:
3234:
3229:
3223:
3221:
3217:
3216:
3213:
3212:
3210:
3209:
3204:
3199:
3193:
3191:
3181:
3180:
3178:
3177:
3172:
3171:
3170:
3160:
3154:
3152:
3142:
3141:
3139:
3138:
3132:
3130:
3117:
3104:
3103:
3100:
3099:
3097:
3096:
3091:
3085:
3083:
3079:
3078:
3076:
3075:
3070:
3065:
3059:
3057:
3051:
3050:
3048:
3047:
3042:
3037:
3035:Minimal change
3031:
3029:
3020:
3012:
3011:
3004:
3003:
2996:
2989:
2981:
2972:
2971:
2969:
2968:
2963:
2957:
2955:
2951:
2950:
2947:
2946:
2944:
2943:
2938:
2933:
2928:
2922:
2920:
2916:
2915:
2913:
2912:
2910:IgA vasculitis
2907:
2901:
2899:
2893:
2892:
2890:
2889:
2888:
2887:
2882:
2870:
2869:
2868:
2855:
2853:
2844:
2838:
2837:
2835:
2834:
2829:
2824:
2818:
2816:
2810:
2809:
2807:
2806:
2801:
2795:
2793:
2787:
2786:
2779:
2778:
2771:
2764:
2756:
2747:
2746:
2743:
2742:
2731:
2720:
2709:
2698:
2687:
2675:
2674:
2672:
2668:
2667:
2664:
2663:
2652:
2641:
2626:
2611:
2595:
2590:
2589:
2587:
2586:Classification
2579:
2578:External links
2576:
2573:
2572:
2528:
2485:
2450:
2407:
2361:
2320:
2291:
2267:10.1186/ar3797
2240:
2213:
2176:(3): 221–232.
2155:
2109:
2072:(3): 315–320.
2051:
2010:
1974:
1938:
1911:
1892:(2): 195–216.
1873:
1846:
1835:(2): 193–202.
1819:
1768:
1744:"Episcleritis"
1734:
1690:
1638:
1592:
1573:(8): 502–505.
1557:
1538:(6): W619–29.
1518:
1454:
1392:
1332:
1286:
1248:
1213:
1159:
1113:
1064:
1063:
1061:
1058:
1057:
1056:
1049:
1046:
1036:(ASN) and the
938:
935:
922:
919:
887:
884:
868:
865:
857:TNF inhibitors
820:bladder cancer
784:Plasmapheresis
767:
766:Severe disease
764:
759:
756:
749:
748:
741:
726:
725:
724:
723:
720:
703:
702:
701:
695:
684:
683:
682:
679:
673:
667:
666:
665:
659:
625:
622:
597:
596:Classification
594:
526:
523:
441:
438:
421:
418:
417:
416:
402:
392:Nervous system
389:
373:
364:
361:cough up blood
346:
337:
336:
335:
328:
303:
286:conjunctivitis
246:. In general,
227:
224:
183:and high-dose
161:can be fatal.
105:
104:
99:
95:
94:
77:
71:
70:
51:
50:
42:
41:
38:
34:
33:
25:
15:
9:
6:
4:
3:
2:
3310:
3299:
3298:Rare diseases
3296:
3294:
3291:
3289:
3286:
3284:
3281:
3279:
3276:
3274:
3271:
3269:
3266:
3264:
3261:
3259:
3256:
3254:
3251:
3250:
3248:
3233:
3230:
3228:
3225:
3224:
3222:
3218:
3208:
3205:
3203:
3200:
3198:
3195:
3194:
3192:
3190:
3186:
3182:
3176:
3173:
3169:
3166:
3165:
3164:
3161:
3159:
3156:
3155:
3153:
3151:
3147:
3143:
3137:
3134:
3133:
3131:
3129:
3125:
3121:
3118:
3116:
3111:
3105:
3095:
3092:
3090:
3087:
3086:
3084:
3080:
3074:
3071:
3069:
3066:
3064:
3061:
3060:
3058:
3056:
3055:Proliferative
3052:
3046:
3043:
3041:
3038:
3036:
3033:
3032:
3030:
3028:
3024:
3021:
3019:
3013:
3009:
3002:
2997:
2995:
2990:
2988:
2983:
2982:
2979:
2967:
2964:
2962:
2959:
2958:
2956:
2952:
2942:
2939:
2937:
2934:
2932:
2929:
2927:
2924:
2923:
2921:
2917:
2911:
2908:
2906:
2903:
2902:
2900:
2898:
2894:
2886:
2883:
2881:
2878:
2877:
2876:
2875:
2871:
2867:
2864:
2863:
2862:
2861:
2857:
2856:
2854:
2852:
2848:
2845:
2843:
2839:
2833:
2830:
2828:
2825:
2823:
2820:
2819:
2817:
2815:
2814:Medium vessel
2811:
2805:
2802:
2800:
2797:
2796:
2794:
2792:
2788:
2784:
2777:
2772:
2770:
2765:
2763:
2758:
2757:
2754:
2741:
2737:
2736:
2732:
2730:
2726:
2725:
2721:
2719:
2715:
2714:
2710:
2708:
2704:
2703:
2699:
2697:
2693:
2692:
2688:
2686:
2682:
2681:
2677:
2676:
2673:
2669:
2662:
2658:
2657:
2653:
2651:
2647:
2646:
2642:
2640:
2636:
2635:
2631:
2627:
2625:
2621:
2620:
2616:
2612:
2610:
2606:
2605:
2601:
2597:
2596:
2593:
2588:
2584:
2568:
2564:
2560:
2556:
2552:
2548:
2544:
2540:
2532:
2524:
2520:
2516:
2512:
2508:
2504:
2500:
2496:
2489:
2481:
2477:
2473:
2469:
2466:(6): 829–31.
2465:
2461:
2454:
2446:
2442:
2438:
2434:
2430:
2426:
2422:
2418:
2411:
2403:
2399:
2395:
2391:
2387:
2383:
2380:(6): 481–93.
2379:
2375:
2368:
2366:
2357:
2353:
2348:
2343:
2340:(6): 655–69.
2339:
2335:
2331:
2324:
2310:on 2014-09-14
2309:
2305:
2301:
2295:
2287:
2283:
2278:
2273:
2268:
2263:
2259:
2255:
2251:
2244:
2228:
2224:
2217:
2209:
2205:
2200:
2195:
2191:
2187:
2183:
2179:
2175:
2171:
2167:
2159:
2151:
2147:
2143:
2139:
2135:
2131:
2127:
2123:
2116:
2114:
2105:
2101:
2097:
2093:
2088:
2083:
2079:
2075:
2071:
2067:
2063:
2055:
2047:
2043:
2038:
2033:
2029:
2025:
2021:
2014:
2006:
2002:
1998:
1994:
1991:(2): 187–92.
1990:
1986:
1978:
1970:
1966:
1962:
1958:
1955:(8): 1101–7.
1954:
1950:
1942:
1926:
1922:
1915:
1907:
1903:
1899:
1895:
1891:
1887:
1880:
1878:
1861:
1857:
1850:
1842:
1838:
1834:
1830:
1823:
1815:
1811:
1806:
1801:
1796:
1791:
1787:
1783:
1779:
1772:
1757:
1753:
1749:
1745:
1738:
1730:
1726:
1722:
1718:
1715:(1): 95–111.
1714:
1710:
1703:
1701:
1699:
1697:
1695:
1686:
1682:
1678:
1674:
1670:
1666:
1662:
1658:
1651:
1649:
1647:
1645:
1643:
1634:
1630:
1626:
1622:
1618:
1614:
1607:
1605:
1603:
1601:
1599:
1597:
1588:
1584:
1580:
1576:
1572:
1568:
1561:
1553:
1549:
1545:
1541:
1537:
1533:
1525:
1523:
1514:
1510:
1505:
1500:
1496:
1492:
1488:
1484:
1480:
1476:
1472:
1465:
1463:
1461:
1459:
1450:
1446:
1441:
1436:
1432:
1428:
1424:
1417:
1415:
1413:
1411:
1409:
1407:
1405:
1403:
1401:
1399:
1397:
1388:
1384:
1380:
1376:
1372:
1368:
1365:(8): 1273–9.
1364:
1360:
1353:
1351:
1349:
1347:
1345:
1343:
1341:
1339:
1337:
1328:
1324:
1320:
1316:
1312:
1308:
1305:(6): 613–16.
1304:
1300:
1293:
1291:
1282:
1278:
1274:
1270:
1267:(1): 91–101.
1266:
1262:
1255:
1253:
1244:
1240:
1236:
1232:
1228:
1224:
1217:
1209:
1205:
1200:
1195:
1191:
1187:
1183:
1179:
1175:
1168:
1166:
1164:
1155:
1151:
1147:
1143:
1139:
1135:
1131:
1127:
1120:
1118:
1109:
1105:
1101:
1097:
1093:
1089:
1086:(3): 248–53.
1085:
1081:
1074:
1072:
1070:
1065:
1055:
1052:
1051:
1045:
1043:
1039:
1035:
1031:
1025:
992:
988:
984:
981:
977:
972:
970:
966:
962:
958:
954:
950:
946:
943:
934:
932:
928:
918:
914:
912:
908:
904:
899:
897:
893:
883:
881:
877:
873:
864:
862:
858:
854:
850:
846:
842:
838:
832:
830:
825:
821:
817:
813:
807:
805:
801:
797:
793:
789:
785:
781:
777:
773:
763:
755:
752:
746:
742:
739:
738:granulomatous
735:
734:
733:
731:
721:
719:
711:
707:
706:
704:
700:
696:
694:
691:
688:
687:
685:
680:
678:
674:
671:
670:
668:
663:
660:
658:
654:
653:
651:
650:
645:
641:
639:
635:
631:
628:In 1990, the
621:
619:
615:
611:
610:blood vessels
607:
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583:granulomatous
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567:thoracoscopic
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511:degranulation
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342:: subglottal
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325:auditory tube
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67:H&E stain
64:
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56:
52:
48:
43:
39:
35:
30:
22:
3278:Rheumatology
3196:
3189:Pauci-immune
3185:Type III RPG
3082:By condition
2872:
2865:
2858:
2851:Pauci-immune
2842:Small vessel
2791:Large vessel
2733:
2722:
2711:
2700:
2689:
2678:
2654:
2643:
2628:
2613:
2598:
2542:
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2501:(2): 202–6.
2498:
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2463:
2459:
2453:
2420:
2416:
2410:
2377:
2373:
2337:
2333:
2323:
2312:. Retrieved
2308:the original
2303:
2294:
2257:
2253:
2243:
2231:. Retrieved
2226:
2216:
2173:
2169:
2158:
2128:(1): 25–36.
2125:
2121:
2069:
2065:
2054:
2030:(4): 453–8.
2027:
2023:
2013:
1988:
1984:
1977:
1952:
1948:
1941:
1929:. Retrieved
1924:
1914:
1889:
1885:
1864:. Retrieved
1859:
1849:
1832:
1828:
1822:
1785:
1781:
1771:
1759:. Retrieved
1747:
1737:
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1708:
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1656:
1619:(1): 39–50.
1616:
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1184:(1): 60–64.
1181:
1177:
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990:
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940:
924:
921:Epidemiology
915:
900:
889:
874:
870:
845:azathioprine
841:methotrexate
833:
808:
794:(as seen in
769:
761:
753:
750:
727:
717:
692:
676:
675:infiltrates
656:
627:
599:
586:inflammation
581:changes and
574:
556:
544:proteinase 3
536:
498:
491:proteinase 3
484:
473:
467:
457:
443:
434:pathogenesis
423:
383:
321:hearing loss
306:Upper airway
282:episcleritis
267:
241:
205:
197:methotrexate
193:azathioprine
170:
163:
121:
117:
113:
109:
108:
84:rheumatology
3146:Type II RPG
3094:Amyloidosis
2680:MedlinePlus
2423:(1): 1–23.
1229:(1): 1–19.
1032:(ACR), the
983:pathologist
849:leflunomide
812:infertility
616:(EGPA) and
550:(a type of
548:neutrophils
546:(cANCA) in
507:endothelium
503:neutrophils
454:giant cells
452:, and many
269:saddle-nose
236:saddle nose
37:Other names
3273:Nephrology
3247:Categories
3124:Type I RPG
3045:Membranous
2702:Patient UK
2656:DiseasesDB
2545:(4): 704.
2314:2016-03-16
2260:(2): 210.
2124:(Review).
1888:(Review).
1831:(Review).
1761:10 January
1748:StatPearls
1711:(Review).
1534:(Review).
1433:: 227–40.
1429:(Review).
1361:(Review).
1301:(Review).
1225:(Review).
1180:(Review).
1128:(Review).
1082:(Review).
1060:References
1042:Nazi Party
861:etanercept
859:, such as
855:are used.
745:vasculitis
590:microscopy
519:arterioles
466:including
446:granulomas
332:gingivitis
264:runny nose
260:nosebleeds
143:nosebleeds
130:granulomas
80:Immunology
63:granulomas
59:vasculitis
55:Micrograph
3253:Arthritis
3110:nephritic
3107:Primarily
3018:nephrotic
3015:Primarily
2919:Ungrouped
2691:eMedicine
2445:222321164
2190:0028-4793
2096:0003-4967
1685:206894936
1495:1469-493X
1387:206849855
1132:(6): 36.
996:English:
969:lymphomas
927:incidence
886:Prognosis
837:rituximab
772:rituximab
758:Treatment
697:red cell
525:Diagnosis
367:Arthritis
353:pneumonia
316:disease:
278:scleritis
177:rituximab
75:Specialty
3089:Diabetic
2735:Orphanet
2696:med/2401
2567:12409848
2559:21372195
2523:25978459
2515:16601443
2480:13275478
2402:12082375
2394:22927039
2356:17684188
2286:22569190
2233:16 March
2208:20647199
2142:24189648
2104:35110333
1931:16 March
1906:28506361
1866:16 March
1841:25791629
1814:36536738
1756:30521217
1729:27888918
1677:22250224
1633:15210387
1552:26587951
1513:31927782
1449:25341007
1379:23606701
1327:36254262
1319:28817388
1281:26611553
1243:25399936
1208:28148583
1154:21560794
1146:27118389
1108:35805200
1100:28306595
1048:See also
942:Scottish
911:deafness
681:cavities
672:nodules,
662:purulent
624:Criteria
606:antibody
579:necrotic
499:In vitro
450:necrosis
426:bacteria
344:stenosis
244:symptoms
234:Typical
212:Japanese
175:such as
166:Genetics
141:include
3220:General
2650:D014890
2609:4A44.A1
2437:7057076
2277:3446448
2229:. WebMD
2199:3137658
2150:8483900
2046:9159539
2005:8129773
1969:2202308
1862:. WebMD
1805:9758322
1663:: e26.
1587:1914353
1504:6956643
1199:6297586
937:History
430:viruses
380:purpura
340:Trachea
323:due to
256:sinuses
159:kidneys
2874:p-ANCA
2860:c-ANCA
2685:000135
2565:
2557:
2521:
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1152:
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1098:
980:German
892:uremia
818:, and
563:biopsy
478:, and
469:PTPN22
420:Causes
293:Kidney
284:) and
266:, and
220:affect
98:Causes
86:
3163:Lupus
2954:Other
2661:14057
2639:446.4
2624:M31.3
2563:S2CID
2519:S2CID
2441:S2CID
2398:S2CID
2146:S2CID
1681:S2CID
1383:S2CID
1323:S2CID
1150:S2CID
1104:S2CID
851:, or
699:casts
577:with
475:CTLA4
413:brain
405:Heart
349:Lungs
155:lungs
151:heart
2724:GARD
2713:NORD
2645:MeSH
2634:9-CM
2555:PMID
2511:PMID
2476:PMID
2433:PMID
2390:PMID
2352:PMID
2334:JAMA
2282:PMID
2235:2014
2204:PMID
2186:ISSN
2138:PMID
2100:PMID
2092:ISSN
2042:PMID
2001:PMID
1965:PMID
1933:2014
1902:PMID
1868:2014
1837:PMID
1810:PMID
1763:2024
1752:PMID
1725:PMID
1673:PMID
1629:PMID
1583:PMID
1548:PMID
1509:PMID
1491:ISSN
1445:PMID
1375:PMID
1315:PMID
1277:PMID
1239:PMID
1204:PMID
1142:PMID
1096:PMID
963:and
925:The
561:, a
428:and
376:Skin
312:and
280:and
214:and
206:The
191:and
157:and
132:and
61:and
3175:IgA
3115:RPG
2740:900
2630:ICD
2615:ICD
2600:ICD
2547:doi
2503:doi
2468:doi
2425:doi
2382:doi
2342:doi
2338:298
2272:PMC
2262:doi
2194:PMC
2178:doi
2174:363
2130:doi
2082:hdl
2074:doi
2032:doi
1993:doi
1957:doi
1894:doi
1833:150
1800:PMC
1790:doi
1717:doi
1665:doi
1661:344
1657:BMJ
1621:doi
1617:117
1575:doi
1540:doi
1536:205
1499:PMC
1483:doi
1435:doi
1367:doi
1307:doi
1269:doi
1231:doi
1194:PMC
1186:doi
1134:doi
1088:doi
989:or
949:BMJ
894:or
774:or
314:ear
310:eye
179:or
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