Authors :
Omkar Jha; Shushant Guragain; Bishow Raj Gyawali; Ajit Kumar Sah; Isha Paudel; Sneha Shah
Volume/Issue :
Volume 9 - 2024, Issue 9 - September
Google Scholar :
https://tinyurl.com/4mtpc3rf
Scribd :
https://tinyurl.com/5746vzvz
DOI :
https://doi.org/10.38124/ijisrt/IJISRT24SEP691
Note : A published paper may take 4-5 working days from the publication date to appear in PlumX Metrics, Semantic Scholar, and ResearchGate.
Abstract :
Introduction:
Immunoglobulin A (IgA) vasculitis, previously
known as Henoch-Schönlein purpura, is an immune
complex-mediated small vessel vasculitis primarily
affecting children. While rare in adults, it can present
with more severe manifestations, particularly involving
the kidneys. This case report details the presentation and
management of adult-onset IgA vasculitis with significant
renal involvement.
Case Report:
A 43-year-old male with a history of bipolar disorder
presented with facial swelling, shortness of breath, and
decreased urine output following an upper respiratory
infection. Initial investigations revealed elevated blood
pressure and renal impairment. Despite supportive
treatment, his condition worsened, leading to a referral to
a tertiary care center. He exhibited symptoms consistent
with IgA vasculitis, including joint pain, rash, and
nephrotic-range proteinuria. The diagnosis was
confirmed through a skin biopsy and 24-hour urine
collection. The patient was treated with intravenous
methylprednisolone, oral prednisone, and an ACE
inhibitor. His renal function improved with this regimen.
Discussion:
Adult-onset IgA vasculitis can present with severe
kidney involvement, including nephrotic-range
proteinuria and elevated serum creatinine, which are
associated with poorer outcomes compared to pediatric
cases. The patient's management, involving
glucocorticoids and an ACE inhibitor, aligns with current
treatment recommendations for significant renal
involvement. Long-term prognosis in adults remains
challenging, with a higher risk of end-stage kidney disease
compared to children. Vigilant monitoring and tailored
treatment strategies are crucial for improving outcomes.
Conclusion:
This case underscores the potential severity of adult-
onset IgA vasculitis and highlights the importance of early
diagnosis and aggressive management to mitigate long-
term renal complications. Ongoing research is necessary
to refine treatment approaches and enhance outcomes for
adults with this condition.
Keywords :
IgA Vasculitis, Henoch-Schönlein Purpura, Proteinuria, Prednisolone, ACE- Inhibitor, Vasculitis.
References :
- Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B: IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects. Autoimmunity reviews. 2015, 14:579-585. 10.1016/j.autrev.2015.02.003
- Byun JW, Song HJ, Kim L, Shin JH, Choi GS: Predictive relapse factors in adults with Henoch-Schönlein purpura. Am J Dermatopathol. 2012, 34:139-144. 10.1097/DAD.0b013e3182157f90
- Oni L, Sampath S: Childhood IgA Vasculitis (Henoch Schonlein Purpura)-Advances and Knowledge Gaps. Frontiers in pediatrics. 2019, 7:257. 10.3389/fped.2019.00257
- Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, González-Gay MA: Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis and rheumatism. 1997, 40:859-864. 10.1002/art.1780400513
- Batu ED, Sarı A, Erden A, et al.: Comparing immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children and adults: a single-center study from Turkey. Scandinavian journal of rheumatology. 2018, 47:481-486. 10.1080/03009742.2018.1448111
- Kang Y, Park JS, Ha YJ, et al.: Differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura. Journal of Korean Medical Science. 2014, 29:198-203. 10.3346/jkms.2014.29.2.198
- Hočevar A, Rotar Z, Ostrovršnik J, et al.: Incidence of IgA vasculitis in the adult Slovenian population. The British journal of dermatology. 2014, 171:524-527. 10.1111/bjd.12946
- Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D: Henoch-Schönlein Purpura in adults: outcome and prognostic factors. Journal of the American Society of Nephrology: JASN. 2002, 13:1271-1278. 10.1097/01.asn.0000013883.99976.22
- McCarthy HJ, Tizard EJ: Clinical practice: Diagnosis and management of Henoch-Schönlein purpura. European journal of pediatrics. 2010, 169:643-650. 10.1007/s00431-009-1101-2
- Kiryluk K, Moldoveanu Z, Sanders JT, et al.: Aberrant glycosylation of IgA1 is inherited in pediatric IgA nephropathy and Henoch-Schönlein purpura nephritis. Kidney international. 2011, 80:79-87. 10.1038/ ki.2011.16
- Chang WL, Yang YH, Wang LC, Lin YT, Chiang BL: Renal manifestations in Henoch-Schönlein purpura: a 10-year clinical study. Pediatric nephrology (Berlin, Germany). 2005, 20:1269-1272. 10.1007/s00467-005-1903-z
- Narchi H: Risk of long-term renal impairment and duration of follow-up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review. Archives of disease in childhood. 2005, 90:916-920. 10.1136/adc.2005. 074641
- KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney international. 2021, 100:S1-s276. 10.1016/j.kint. 2021.05.021
- Ozen S, Marks SD, Brogan P, et al.: European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology (Oxford, England). 2019, 58:1607-1616. 10.1093/rheumatology/kez041
- Hackl A, Becker JU, Körner LM, et al.: Mycophenolate mofetil following glucocorticoid treatment in Henoch-Schönlein purpura nephritis: the role of early initiation and therapeutic drug monitoring. Pediatric nephrology (Berlin, Germany). 2018, 33:619-629. 10.1007/s00467-017-3846-6
- Maritati F, Fenoglio R, Pillebout E, et al.: Brief Report: Rituximab for the Treatment of Adult-Onset IgA Vasculitis (Henoch-Schönlein). Arthritis & rheumatology (Hoboken, NJ). 2018, 70:109-114. 10.1002/art.40339
- Shi D, Chan H, Yang X, et al.: A meta-analysis: Risk factors associated with IgA vasculitis with nephritis (Henoch-Schönlein purpura nephritis) progressing to unfavorable outcomes. PloS one. 2019, 14:e0223218. 10.1371/journal.pone.0223218
- Coppo R, Andrulli S, Amore A, et al.: Predictors of outcome in Henoch-Schönlein nephritis in children and adults. American journal of kidney diseases: the official journal of the National Kidney Foundation. 2006, 47:993-1003. 10.1053/j.ajkd.2006.02.178
- Shrestha S, Sumingan N, Tan J, Alhous H, McWilliam L, Ballardie F: Henoch Schönlein purpura with nephritis in adults: adverse prognostic indicators in a UK population. QJM: monthly journal of the Association of Physicians. 2006, 99:253-265. 10.1093/qjmed/hcl034
Introduction:
Immunoglobulin A (IgA) vasculitis, previously
known as Henoch-Schönlein purpura, is an immune
complex-mediated small vessel vasculitis primarily
affecting children. While rare in adults, it can present
with more severe manifestations, particularly involving
the kidneys. This case report details the presentation and
management of adult-onset IgA vasculitis with significant
renal involvement.
Case Report:
A 43-year-old male with a history of bipolar disorder
presented with facial swelling, shortness of breath, and
decreased urine output following an upper respiratory
infection. Initial investigations revealed elevated blood
pressure and renal impairment. Despite supportive
treatment, his condition worsened, leading to a referral to
a tertiary care center. He exhibited symptoms consistent
with IgA vasculitis, including joint pain, rash, and
nephrotic-range proteinuria. The diagnosis was
confirmed through a skin biopsy and 24-hour urine
collection. The patient was treated with intravenous
methylprednisolone, oral prednisone, and an ACE
inhibitor. His renal function improved with this regimen.
Discussion:
Adult-onset IgA vasculitis can present with severe
kidney involvement, including nephrotic-range
proteinuria and elevated serum creatinine, which are
associated with poorer outcomes compared to pediatric
cases. The patient's management, involving
glucocorticoids and an ACE inhibitor, aligns with current
treatment recommendations for significant renal
involvement. Long-term prognosis in adults remains
challenging, with a higher risk of end-stage kidney disease
compared to children. Vigilant monitoring and tailored
treatment strategies are crucial for improving outcomes.
Conclusion:
This case underscores the potential severity of adult-
onset IgA vasculitis and highlights the importance of early
diagnosis and aggressive management to mitigate long-
term renal complications. Ongoing research is necessary
to refine treatment approaches and enhance outcomes for
adults with this condition.
Keywords :
IgA Vasculitis, Henoch-Schönlein Purpura, Proteinuria, Prednisolone, ACE- Inhibitor, Vasculitis.