A Detailed Analysis of Renal Involvement and Prognosis in Adult-Onset Immunglobulin (Ig)-A Vasculitis

Öz

Immunglobulin (Ig)- A vasculitis is seen more common in children characterized with benign course, whereas adult-onset form is relatively rare but may be associated with severe organ involvement and worse prognosis. Our aim in this study is to retrospectively evaluate clinical features, detailed analysis of renal involvement, longterm renal outcome and overall prognosis in adult-onset IgA vasculitis patients followed in our center. 52 cases fullfilling selection criterias were included in this study. Majority of patients were male (37 vs 15, 71,2% vs 28.8%) and mean age was calculated 45 ± 18 years. Among 20 patients who had renal involvement, renal biopsy was performed in 12 (23,1%), consistent with IgA nephropathy. Chronic kidney disease (CKD) developed in 5, and among those, hemodialysis was needed in 3 at least once through entire following period. Relaps was seen in 9 (17,3%) with the sites of distribution as following; 5 renal, 3 skin and 1 gastrointestinal. Acute kidney injury (50% vs 10% p:0,002) and nephrotic range proteinuria (75% vs 32,5% p:0,028) at admission were found significantly higher in patients at advanced age (>65). CKD development (33,3% vs 2,5%, p<0,001) and overall mortality were also higher (41,7% vs 5% p:0,001) in this group. Mortality was seen in 7 patients and 71,4% of them had acute kidney injury at the time of first admission (p<0,001). In our study, we have found that advanced age (>65) and renal involvement seem to be significant risk factors for the development of CKD and overall mortality, consistent with the literature. Therefore, these patients should be monitored closely.

Anahtar Kelimeler

• IgA vasculitis
• renal involvement
• advanced age
• prognosis

Erişkin Başlangıçlı İmmunglobulin (Ig)-A Vaskülitinde Ayrıntılı Renal Tutulum Sonuçları ve Prognoz

Öz

İmmunglobulin-(Ig) A vasküliti çocukluk çağında daha sık ve benign seyir gösterirken, erişkin başlangıç daha nadirdir ve ağır seyretmektedir. Amacımız, merkezimizdeki IgA vaskülitli erişkin hastaların ayrıntılı klinik özellikleri, uzun dönem renal tutulum sonuçlarını ve prognozlarını retrospektif olarak belirlemekti. Çalışmaya IgA vasküliti tanı kriterlerini karşılayan 52 hasta alındı. Hastaların çoğunluğu erkek (37 vs 15, %71,2 vs %28.8) ve ortalama yaş 45 ± 18 yıl olarak bulundu. Renal tutulumu olan 20 hastanın, 12’sine (%23,1) renal biyopsi yapılmış ve IgA nefropatisi ile uyumlu saptanmıştır. 5 hastada kronik renal yetmezlik (KRY) gelişti ve bunların 3’ünde hemodiyaliz desteğinin en az bir kez gerektiği görüldü. 9 (%17,3) hastada relaps gözlendi (5’i renal, 3’ü cilt ve 1’i gastrointestinal). İleri yaş (>65) olanlarda başvuru sırasında akut böbrek yetmezliği (%50 vs %10 p:0,002) ve nefrotik düzeyde proteinüri (%75 vs %32,5 p:0,028) daha yüksek oranda tespit edildi. Bu grupta KRY gelişme riski (%33,3 vs %2,5 p<0,001) ve ölüm oranı (%41,7 vs %5 p:0,001) daha yüksekti. Takipleri sırasında 7 hasta ölmüştü. Bu hastaların başvuru semptomları incelendiğinde başvuru anında 5’inde (%71,4) akut böbrek yetmezliği mevcuttu (%71,4 vs %11,1 p<0,001). Sonuç olarak çalışmamızda ileri yaş (>65) ve renal tutulum, KRY gelişimi ve ölüm için önemli bir kriter olarak tespit edilmiştir. Bu nedenle ileri yaşta başlayan ve renal tutulumu olan hastalar KRY gelişimi ve kötü prognoz açısından dikkatle takip edilmelidirler

Anahtar Kelimeler

• IgA vasküliti
• renal tutulum
• ileri yaş
• prognoz

Kaynakça

1. Gardner-Medwin JM, Dolezalova P, Cummins C, et al. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. The Lancet. 2002;360:1197-202.
2. Yaseen K, Herlitz LC, Villa-Forte A. IgA Vasculitis in Adults: a Rare yet Challenging Disease. Curr Rheumatol Rep. 2021;23:50.
3. Komatsu H, Fujimoto S, Maruyama S, et al. Distinct characteristics and outcomes in elderly-onset IgA vasculitis (Henoch-Schönlein purpura) with nephritis: Nationwide cohort study of data from the Japan Renal Biopsy Registry (J-RBR). PloS one. 2018;13:e0196955.
4. Ozen S, Pistorio A, Iusan SM, et al; Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69:798-806.
5. Audemard-Verger A, Terrier B, Dechartres A, et al; French Vasculitis Study Group. Characteristics and management of IgA vasculitis (Henoch-Schönlein) in adults: data from 260 patients included in a French multicenter retrospective survey. Arthritis Rheumatol. 2017;69:1862-70.
6. Inker LA, Astor BC, Fox CH, et al. KDOQI US commentary on the 2012 KDIGO clinical practice guideline for the evaluation and management of CKD. Am J Kidney Dis. 2014;63:713-35.
7. Van de Perre E, Jones RB, Jayne DRW. IgA vasculitis (Henoch-Schönlein purpura): refractory and relapsing disease course in the adult population. Clin Kidney J. 2021;14:1953-60.
8. Villatoro-Villar M, Crowson CS, Warrington KJ, et al. Clinical Characteristics of Biopsy-Proven IgA Vasculitis in Children and Adults: A Retrospective Cohort Study. Mayo Clin Proc. 2019;94:1769-80.

9. Calvo-Rio V, Loricera J, Mata C et al. Henoch-Schonlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center. Medicine (Baltimore) 2014;93: 106–13.
10. Selewski DT, Ambruzs JM, Appel GB, et al. Clinical characteristics and treatment patterns of children and adults with IgA nephropathy or IgA vasculitis: findings from the CureGN study. Kidney Int Rep. 2018;3:1373–84.
11. Coppo R, Andrulli S, Amore A, et al. Predictors of outcome in Henoch-Schonlein nephritis in children and adults. Am J Kidney Dis 2006; 47: 993–1003
12. Pillebout E, Thervet E, Hill G et al. Henoch-Schonlein purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002;13:1271–78
13. Audemard-Verger A, Pillebout E, Guillevin L, et al. IgA vasculitis (Henoch-Scho¨ nlein purpura) in adults: diagnostic and therapeutic aspects. Autoimmun Rev. 2015;14:579–85
14. Pillebout E, Alberti C, Guillevin L, et al. Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schonlein purpura. Kidney Int. 2010; 78: 495–502
15. Rauen T, Eitner F, Fitzner C et al. Intensive supportive care plus immunosuppression in IgA nephropathy. N Engl J Med. 2015;373:2225–36