Impact of aging on phenotype and prognosis in IgA vasculitis

Abstract

Objectives Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis most frequently benign in children while more severe in adults. We aimed to study the impact of age on presentation and outcome of adult IgAV. Methods We conducted a nationwide retrospective study including 260 IgAV patients. Patients were divided into four quartiles according to the age at IgAV diagnosis: <36, 36 ≤ age < 52; 52 ≤ age < 63 and ≥63 years. Comparison of presentation and outcome were performed according to age of disease onset. Results Mean age at diagnosis was 50.1 (18) years and 63% were male. IgAV diagnosed in the lowest quartile of age was associated with more frequent joint (P < 0.0001) and gastrointestinal involvement (P = 0.001). In contrast, the oldest patients had more severe purpura with necrotic lesions (P = 0.001) and more frequent renal involvement (P < 0.0001), with more frequent haematuria, renal failure, higher urine protein excretion and more frequent tubulointerstitial lesions. Patients were treated similarly in all groups of age, and clinical response and relapse rates were similar between groups. In the 127 treated patients with follow-up data for >6 months, clinical response and relapse rates were similar between the four groups. Median follow-up was of 17.2 months (9.1–38.3 months). Renal failure at the end of follow-up was significantly more frequent in the highest quartile of age (P = 0.02), but the occurrence of end-stage renal disease was similar in all groups. Last, overall and IgAV-related deaths were associated with increase in age. Conclusion Aging negatively impacts the severity and outcome of IgAV in adults. Younger patients have more frequent joint and gastrointestinal involvement, while old patients display more frequent severe purpura and glomerulonephritis.

Navigating the initial diagnosis and management of adult IgA vasculitis: A review

“…IgAV. 45,48 ESRD, End-stage renal disease; IgAV, IgA vasculitis; IgAV-N, IgA vasculitis nephropathy.…”

“…IgAV-associated joint involvement in adults has been reported in 30% to 60% of cases. 44,45 It is transient, migratory, and overall nondestructive. 20,21 The knees and ankles are the most commonly affected joints.…”

Cutaneous vasculitis; An algorithmic approach to diagnosis

“…IgA vasculitis is more severe in adults. While joint and gastrointestinal involvement is more common in younger patients, severe purpura and glomerulonephritis are more common in older patients ( 15 ). IgA vasculitis is the most common vasculitis of childhood.…”

IgA vasculitis update: Epidemiology, pathogenesis, and biomarkers

“…In addition, recent studies have shown that patients with tubular or interstitial lesions and glomerulosclerosis are associated with poor renal outcomes, and patients with both nephritic and nephrotic syndrome have the highest risk for progression to ESRD ( 6 – 9 ). Adult patients face a poorer prognosis than children, leaning more towards a protracted and chronic course, of who the incidence of ESRD is up to 32% ( 10 – 12 ).…”