Characterization of Adult-Type IgA Vasculitis (Henoch-Schönlein Purpura): A Retrospective Study of 122 Cases

Abstract

The clinical features of adult-type IgA vasculitis have not been well characterized. To analyze the characteristics of IgA vasculitis in adults, patients diagnosed with IgA vasculitis based on EULAR/PRINTO/PRES criteria (2012) in our institution between 2003 and 2012 were studied, comprising 85 adults (age ≥ 21 years) and 37 pediatric patients (≤ 20 years). Compared with pediatric cases, adult disease showed significantly higher serum C-reactive protein and IgA values, a lower percentage of cases was associated with infections (56.5% vs 89.2%, P < 0.001) but there was a greater range of infections affecting different tissues and organs, and there was occasional cases with malignancy (8.2%) including four cases of lung carcinoma and three with hematological disorders. The skin lesions in adults tended to be widely distributed on the abdomen and waist (15.3% vs 2.7%, P = 0.045). Adult cases were associated with greater renal involvement, as evidenced by proteinuria, hematuria and/or urinary casts, compared with the pediatric group (76.2% vs 48.6%, P = 0.003) and disease recalcitrance was also significantly higher (38.8% vs 18.9%, P = 0.031). Examination of the serum levels of immunoglobulins in adults showed that a sole increase in IgA was associated with renal and gastrointestinal manifestations, but this was not seen in cases with concurrent increases of IgA and IgG or IgA, IgG and IgM. Although the retrospective nature of the study is a limitation, it identified possible associations with the wide range of infections, more severe renal damage, and malignancy in adult IgA vasculitis.

Clinical Characteristics of Biopsy-Proven IgA Vasculitis in Children and Adults: A Retrospective Cohort Study

“…14,18,21,22,31 Although the distribution of palpable purpura was similar between adult-and childhood-onset IgAV in the current series, adults had more severe lesions, with ulcerative or hemorrhagic skin findings detected in 10.9% compared with only 1.4% of children. Although few studies have reported the presence of such skin lesions among patients with IgAV, Hitomi et al 31 identified ulcers or hemorrhagic bullae in 14.1% of their adult population compared with 5.4% of children. The presence of ulcerative lesions has been reported by other groups evaluating only adult-onset IgAV, with frequencies between 13% and 35%.…”

Concurrent pulmonary coccidioidomycosis and immunoglobulin A vasculitis with hemorrhagic bullae

“…The presence of hemorrhagic bullous lesions in IgAV has been reported to occur in 10.9%-14.1% of biopsy-proven adult IgAV cases. 4 , 6 Biopsied specimens of hemorrhagic lesions demonstrate histopathologic features of red blood cell extravasation, neutrophilic infiltration, and edema.…”

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

“…In a recent study, infections such as pneumonia, pyelonephritis, epididymitis, and cellulitis were found to be present in adults 4 weeks before their IgAV diagnosis. 13 Obtaining a history of previous illness or infections is essential, especially because patients may have a less complicated course of IgAV if it has been provoked by an infection that resolves, as opposed to idiopathic IgAV.…”

“…7 Recalcitrant disease is significantly more common than in children, especially in adults with renal manifestations. 13 Spontaneous remission more likely in patients who have milder disease. 44…”

“…42 Others have proposed checking plasma factor XIII activity and fecal occult blood test in patients with abdominal symptoms. 13 If there is no GI involvement, patients should monitor for these symptoms and immediately report them if identified. Abdominal involvement is more common once the disease is fully established.…”