Immunoglobulin a (IgA) Vasculitis in the Elderly

IgA vasculitis is a small vessel vasculitis mediated by the deposition of IgA immune complexes. It mostly occurs in children and is rare in adults, with increased severity and mortality in the latter. Its aetiology remains largely unknown, and its prognosis depends primarily on the extent of renal involvement. We present the case of a 71-year-old woman with purpuric lesions in both lower and upper limbs associated with fever, abdominal pain, vomiting and blood in her stools for the past month. The patient was diagnosed with IgA vasculitis and the full systemic involvement (renal, dermatological, intestinal, and cerebral) of the disease was identified with excellent response to parenteral corticotherapy.


Introduction
Immunoglobulin A vasculitis (IgAV), previously named Henoch-Schonlein Purpura (HSP), is the most common systemic vasculitis of childhood and is two to 33 times more common in children than in adults. The estimated annual incidence is 0.8-1.8/100,000 for adults [1]. Most studies show a male-to-female ratio of 1.2:1 to 1.8:1. Rarely occurring in summer months, IgAV primarily occurs in the fall, winter, and spring, which can be possibly explained by the association of IgAV with infections. Though the underlying cause of IgAV remains unknown, it is thought that it represents an immune-mediated small vessel vasculitis that could be triggered by a variety of antigens [2]. About half of the cases of IgAV are preceded by an upper respiratory tract infection, primarily in children. Other triggers have been studied such as infectious agents, vaccination, and insect bites. The most consensual diagnostic criteria were developed by the European Alliance of Association for Rheumatology (EULAR) and the Paediatric Rheumatology European Society (PRES), being posteriorly validated by the Paediatric Rheumatology International Trials Organization (PRINTO). The mandatory criterion for diagnosis is the presence of purpura or petechiae, most predominant in the lower limbs, without coagulopathy or thrombocytopenia. One or more criteria are also necessarykidney involvement, such as hematuria or proteinuria, abdominal pain, usually diffuse and of acute onset, leukocytoclastic vascultitis or proliferative glomerulonephritis, with predominant IgA deposits, and arthritis or arthralgia (acute onset) [3].

Case Presentation
A 71-year-old Caucasian woman presented to the Emergency Department (ED) with a month-old polyarthralgia/myalgia and palpable purpuric lesions on the lower and upper limbs (Figures 1, 2).    (Figure 3).

FIGURE 3: Glomerular IgA staining by immunofluorescence microscopy
Due to the bloody stools and abdominal pain an endoscopic study was performed, which revealed multiple ulcerations on segment II of duodenus, compatible with peptic duodenitis; colonoscopy was normal. A brain computer tomography scan (CT) was also performed due to signs of early dementia with abnormal behaviour such as social disinhibition, which showed lesions compatible with microangiopathic insult. Due to the absence of improvement of the skin lesions with topic corticotherapy, a skin biopsy was also performed confirming the presence of IgA deposits.
The diagnosis of IgA vasculitis was assumed, and the patient was medicated with prednisolone 1mg/kg/day, with significant improvement of kidney function and urinary sediment, as well as skin lesions and behavioural issues. After a 38-day admission, the patient was discharged with an SCr of 0.79mL/dL, returning to pre-admission values. She was discharged with Internal Medicine and Nephrology follow-up, with great response to therapy after six months and one year.

Discussion
By the EULAR/PRINTO/PRESS criteria, the patient presented all criteria: purpuric lesions (mandatory criteria); abdominal pain with gastrointestinal bleeding; histopathologic documentation of IgA deposits; arthralgia and renal involvement with proteinuria and hematuria [3]. Having all the criteria is an unusual presentation, with central nervous system involvement being rare but more common in adults and patients with severe forms of the disease [4]. Adults frequently have a worse prognosis, often associated with renal involvement. Age over 65 years, nephrotic proteinuria, acute kidney injury, and hematuria are usually associated with the worst prognosis [5,6]. Systemic corticotherapy should be considered for stabilization of the disease, improving not only quality of life but also disease prognosis. Early corticosteroid treatment significantly reduced the odds of developing the persistent renal disease [7]. However, the complications of steroid therapy, especially in the elderly, must the taken into consideration such as increased susceptibility to infection and femoral neck fractures [8]. Impaired renal function in the elderly could result in higher blood concentrations of steroids, and prolonged therapy could result in immune suppression [9]. Steroid therapy should be short and in the lowest dose needed to reduce the possibility of side effects.

Conclusions
IgAV in adults, particularly in the elderly, is an uncommon and underdiagnosed condition. This case report, with all the EULAR/PRINTO/PRESS criteria, is a rare case, with most cases only presenting two or three criteria. Even though this case presented with clinical features associated with a poorer prognosis, clinical suspicion and the early corticotherapy treatment were the key to the overall success. In conclusion, this report highlights that when examining elderly patients with palpable purpura and abdominal pain, the possibility of IgAV should be considered.

Additional Information Disclosures
Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.