Adult-onset IgA vasculitis (Henoch-Schönlein): Update on therapy

Maritati, Federica; Canzian, Alice; Fenaroli, Paride; Vaglio, Augusto

doi:10.1016/j.lpm.2020.104035

Cited by 38 publications

(54 citation statements)

References 64 publications

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“…IgA vasculitis, also known as Henoch-Schonlein purpura, is a rare disease in the adult population. Prevalence rates in the adult population have varied in literature but are as rare as 0.1 in 100,000 [ 1 ]. In the geriatric population, cases are even more sparse with true prevalence rates being relatively unknown.…”

Section: Introductionmentioning

confidence: 99%

Paraneoplastic IgA Vasculitis in an Older Adult With Metastatic Lung Adenocarcinoma

Farzam

Syed

2021

Cureus

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Immunoglobulin A (IgA) vasculitis is a rare disease in the older adult patient population. It is often triggered by an underlying etiology such as infection or malignancy. Paraneoplastic cases have been reported in the geriatric population in literature but the exact prevalence is unknown. Diagnosis requires satisfaction of both clinical and laboratory criteria, with additional emphasis on finding an underlying etiology. Treatment is focused on supportive care and may include immunosuppressive therapy. However, targeting the primary cause is also important for improving outcomes. Our patient in this report presented with a novel case of paraneoplastic IgA vasculitis that likely occurred secondary to lung adenocarcinoma.

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Section: Introductionmentioning

confidence: 99%

Paraneoplastic IgA Vasculitis in an Older Adult With Metastatic Lung Adenocarcinoma

Farzam

Syed

2021

Cureus

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“…A randomized controlled trial about intravenous cyclophosphamide (IVCY) demonstrated that the addition of cyclophosphamide to steroid administration provides no benefit compared with steroid administration alone [ 11 ]. On the other hand, there have been a few reports that IVCY is effective for severe HSP nephritis [ 12 ]. IVCY is effective for other types of systemic vasculitis, such as lupus nephritis and Wegener granulomatosis, with renal involvement [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and management of Henoch-Schonlein purpura in Indonesian elderly with severe complication: A rare case

Putri

Awalia

2022

Annals of Medicine &Amp; Surgery

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“…The clinical manifestations of IgAV in adults can vary significantly from its monophasic childhood presentation. The common spectrum of disease includes cutaneous purpura, arthralgia (61%), acute enteritis (48–53%) and glomerulonephritis (45–85%) [ 4 , 8 , 9 ]. Symmetric palpable purpura is nearly constant in patients [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Renal complications are a major determinant of long-term morbidity and mortality of IgAV [ 7 ]. In patients with kidney involvement, 11% will progress to end-stage renal disease (ESRD) [ 9 ]. Poor prognosis factors are impaired renal function at baseline, proteinuria >1 g/day at disease onset, macroscopic hematuria, hypertension, and persistent proteinuria >1 g/day during follow-up [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…In patients with kidney involvement, 11% will progress to end-stage renal disease (ESRD) [ 9 ]. Poor prognosis factors are impaired renal function at baseline, proteinuria >1 g/day at disease onset, macroscopic hematuria, hypertension, and persistent proteinuria >1 g/day during follow-up [ 9 ]. Up to 50% of such patients will progress of ESRD over 10 years.…”

Section: Discussionmentioning

confidence: 99%

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A Case Series of Adult Patients Diagnosed with IgA Vasculitis Requiring Systemic Immunosuppression

Cai

Phipps

et al. 2021

Am J Case Rep

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Case series Patients: Female, 31-year-old • Male, 43-year-old • Female, 64-year-old Final Diagnosis: IgA vasculitis Symptoms: Abdominal pain • haematuria • hemoptysis • joint pain • proteinuria • rash Medication: — Clinical Procedure: — Specialty: Immunology Objective: Rare disease Background: IgA vasculitis (IgAV) is a rare and potentially life-threatening small-vessel vasculitis in adults. The disease course is often more severe than its childhood counterpart. The disease is noted for its heterogeneous presentation with varying severity. There are no current treatment guidelines for severe multi-organ involvement of IgAV. The treatment approaches based on the clinical discretion of treating doctors remain controversial, especially regarding the role, duration, and type of immunosuppression. Case Reports: We present 3 cases of severe multi-organ IgAV encountered at our tertiary referral center between 2016 and 2021, which were treated with different immunosuppression regimens, including combination of systemic corticosteroids, oral immunosuppressants (azathioprine, mycophenolate, and sirolimus), rituximab, and cyclophosphamide. In these patients, IgAV presented differently but were all organ-threatening or life-threatening in nature. IgAV in all patients responded to therapies; however, infection complicating underlying comorbidities was the cause of death in 1 patient and the cause of comorbidities in the other 2. Other treatment-related complications included weight gain, adrenal insufficiency, and secondary hypogammaglobulinemia. Conclusions: IgAV can be a polyphasic and a potentially challenging severe organ-threatening disease to treat in adults. The outcomes presented here highlight the morbidity and substantial risks involved in treating complex IgAV patients. Early use of biologics may have a role in preventing treatment-related toxicity. Further studies on IgAV in adults are urgently needed.

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Adult-onset IgA vasculitis (Henoch-Schönlein): Update on therapy

Cited by 38 publications

References 64 publications

Paraneoplastic IgA Vasculitis in an Older Adult With Metastatic Lung Adenocarcinoma

Paraneoplastic IgA Vasculitis in an Older Adult With Metastatic Lung Adenocarcinoma

Diagnosis and management of Henoch-Schonlein purpura in Indonesian elderly with severe complication: A rare case

A Case Series of Adult Patients Diagnosed with IgA Vasculitis Requiring Systemic Immunosuppression

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