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CASE REPORT
Adult onset of Immunoglobulin A vasculitis – A case report
Shusuke Yagi1,2,3, Itsuro Endo1,4, Taichi Murakami5, Tetsuya Hida1,6, Yousuke Yamamoto1, Tomohiro Soga1,3,7,Takayuki Ise2, Kenya Kusunose2, Koji Yamaguchi2, Daiju Fukuda2, Hirotsugu Yamada2, Takeshi Soeki2,
Tetsuzo Wakatsuki2, Shinji Kawahito1,3,7, and Masataka Sata2
Shikoku Central Hospital, Shikokuchuo, Ehime, Japan, 2Department of Cardiovascular Medicine, Tokushima University Graduate School ofBiomedical Sciences, Tokushima, Japan, 3Department of Community Medicine and Human Resource Development, Tokushima University
Graduate School of Biomedical Sciences, Tokushima, Japan, 4Department of Hematology, Endocrinology and Metabolism, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan, 5Department of Nephrology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan, 6Department of Dermatology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan,
7
Department of Anesthesiology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
1

Abstract : Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, primarily occurs
during childhood between the ages of 3 and 15 years and is the most common form of systemic vasculitis in children ; its occurrence in adults has been rarely reported. Such low incidence could be attributable to either under-diagnosis or misdiagnosis. Thus, not only pediatricians but also physicians should be able to diagnose IgAV
accurately to manage the patients appropriately and avoid its associated complications. In addition, treatmentof adult onset IgAV with renal involvement has not been fully established yet. We describe here a case of adultonset IgAV complicated by proteinuria and pharyngitis, which was cured by no specific treatment. J. Med. Invest.
66 : 344-346, August, 2019
Keywords : Henoch-Schönlein purpura, palpable purpura, adult

INTRODUCTION
Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (1), primarily occurs during childhood
between the ages of 3 and 15 years and is the most common formof systemic vasculitis in children, with it being diagnosed in over
90% of systemic vasculitis cases (1, 2, 3) ; its occurrence in adults
has been rarely reported (4). Such low incidence could be attributable to either under-diagnosis or misdiagnosis.
Adult onset IgAV is known to be associated with worse renal
outcomes in contrast to those in children, although the clinicalmanifestations have not been fully characterized (5, 6). Thus, notonly pediatricians but also physicians should be able to diagnose
IgAV accurately to manage the patients appropriately and avoid
its associated complications. In addition, treatment of adult onset
IgAV with renal involvement has not been fully established yet.
To this end, we describe here a case of adult onset IgAV complicated by proteinuria and pharyngitis, which was cured by nospecific treatment.

122/68 mmHg, pulse, 98 per minute, and body temperature,
37.8℃. Blood examination indicated systemic inflammation
with elevated white blood cells, 11900/µL and fibrinogen, 490
mg/dL, with a positive test for anti-streptolysin O antibody,

CASE
A 20-year-old man with a history of bronchial asthma presented at our hospital with nausea and had noticed skin eruptions on
the lower extremities for the past 3 days (Figures 1A). He also
had a sore throat for the past 5 days and fatigue in the knees andlower legs. Physical examination of the patient demonstrated
palpable purpuric eruptions on the lower extremities (Figure1) and a slight reddish throat. The patient’s blood pressure wasReceived for publication December 6, 2018 ; accepted May 9, 2019.
Address correspondence and reprint requests to Shusuke Yagi, MD,PhD, Department of Cardiovascular Medicine, Tokushima University
Graduate School of Biomedical Sciences, Tokushima, 3-18-15 Kuramoto-cho, Tokushima 770-8503, Japan and Fax : +81-88-633-7894.

Figure 1. A : Images of palpable purpuric eruptions on the lower
extremities (A : front, B: back, C : close image of a purpuric eruption)

The Journal of Medical Investigation Vol. 66 2019

The Journal of Medical Investigation Vol. 66 August 2019suggesting bacterial infection with Streptococcus pyogenes, resulting in pharyngitis. Serum level of creatinine (0.7 mg/dL) was
normal ; however, urinalysis revealed proteinuria of 1+ without
microscopic hematuria. His serum IgA levels were not elevated(317 mg/dL ; normal range, 110–410 mg/dL). Factor XIII level
was decreased to 43% (normal range, 70–140%). A skin biopsy
showed inflammatory infiltrates around the superficial bloodvessels but no nuclear dust and focal fibrinoid necrosis. Direct
immunofluorescent staining of IgA showed IgA deposition in thevascular media indicating a diagnosis of IgAV.
According to the guidelines, we diagnosed him with possible
IgAV due to palpable purpura with the biopsy showing granulocytes in the walls of small arterioles and venules, arthritis/arthralgia (acute onset), and renal involvement (proteinuria)
although he was only 20 years of age and had not experienced
any acute abdominal pain (7, 8).
He was administered 500 mg/day of acetaminophen. In 14
days, his symptoms and purpura completely disappeared, and hestopped visiting the hospital for follow-up. He reports feeling well
one month after his visiting.

Figure 2. Microscopic findings of skin biopsy (hematoxylin and eosinstaining) showing inflammatory infiltrate around the superficial
blood vessels (A : low magnified image, B : high magnified image).
Direct immunofluorescent staining showing IgA positive cells in thevascular media (Green : IgA deposition ; Blue : nucleus stained with4',6-diamidino-2-phenylindole)

345

DISCUSSION
IgAV is a type of small vessel vasculitis mediated by IgA-immune complex deposition, which is characterized by the clinicaltetrad of non-thrombocytopenic palpable purpura, abdominal
pain, arthritis, and renal involvement, and is primarily a childhood disease. In a population-based study, the annual incidence
was reported to be approximately 10–20 per 100,000 in children < 17 years of age, with a peak incidence of 70 per 100,000
in children between the ages of four to seven years (4, 9, 10). In
contrast, IgAV is less common in adults (2.2 per 100,000) (11),although the annual incidence of IgAV in the young adults orelderly has not been completely elucidated because of heterogeneity of the study population. Recent retrospective studies showedthat 25 to 30 percent of patients with IgAV were adults (5, 6),indicating that adult onset IgAV might be under-diagnosed ormisdiagnosed due to its rarity.
Adult onset IgAV is associated with an increased risk fordeveloping significant renal involvement, including higher frequency of nephrotic syndrome, hypertension, an elevated serum
creatinine level, and end-stage renal disease compared with thepediatric onset IgAV (12). In particular, elderly patients wereassociated with a low incidence of prior infection and presenceof arthritis and increased incidence of hypertension and highserum IgA levels (13, 14).
Typically, the onset of disease in pediatric patients is precededby an upper respiratory tract infection (8). The patient experienced vasculitis symptoms 2 days after the prior infection. Theincubation period may be shorter than that in usual cases. IgAV
follows an infection in two-thirds of the cases, with disease onset
at an average of 3–10 days after the start of the infection symptoms (15). The incubation period may depend on the patients’
health condition and bacterial virulence.
The decrease in the Factor XIII levels was associated with anincreased severity of complications, including nephritis and gastrointestinal involvement, and increase in the Factor XIII levels
was associated with the rate of recovery (16). Factor XIII may
be degraded by proteases from leukocytes or consumed aroundthe affected vessels, resulting in decreased Factor XIII activity
in IgAV patients (17). Thus, the Factor XIII level can serve as arisk assessment biomarker for IgAV. However, the difference inthe degree of Factor XIII deficiency between adult and pediatricpatients with IgAV has not been elucidated.
The diagnosis of IgAV with renal involvement is typically
based on the clinical presentation. IgAV is confirmed on thedetection of IgA deposition in the skin or kidney by immunofluorescence microscopy. Differential diagnosis of palpable purpura
includes other systemic autoimmune diseases (hypersensitivity
vasculitis, cryoglobulinemia, systemic lupus erythematosus, andthrombocytopenic purpura). In this case, IgAV was confirmedby skin biopsy, although we did not perform clinical laboratory
tests, including testing for autoantibodies. A renal biopsy canbe performed to establish the diagnosis ; however, this invasive
procedure is considered only for patients with uncertain diagnosis or those with more severe renal involvement (e.g., proteinuria
greater than 1 g/day and/or renal function impairment ; www.
uptodate.com).
In contrast to other types of systemic vasculitis, IgAV is
self-limiting and most patients recover from it spontaneously.
Thus, the management primarily involves supportive care andincludes adequate hydration, rest, and symptomatic relief of painin the majority of cases. There has been no report evaluating thetreatment approaches for adult onset IgAV, and the use of glucocorticoids or immunosuppressants is controversial based on theevidence from studies involving children (3). It has been reported
that glucocorticoid therapy increases the rate of resolution of the346

S. Yagi, et al. Adult onset IgAV

arthritis and abdominal pain ; however, it does not appear toprevent recurrence of the disease (18). Thus, routine glucocorticoid therapy for patients with IgAV to treat symptoms or prevent
renal or gastrointestinal complications is not recommended.
Glucocorticoid therapy may be recommended only for patientswith nephrotic syndrome and with symptoms significant enoughto affect their oral intake, interfere with their ability to ambulate
and perform activities of daily living, and/or require hospitalization (www.uptodate.com).
Altogether, we presented a case of adult-onset IgAV complicated by proteinuria and pharyngitis. We conclude that physicians should be aware of IgAV in the differential diagnoses ofleg purpura not only in children but also adults, especially inpatients with upper respiratory tract infection. Palpable purpura following upper respiratory tract infection could be a cue tosuspect IgAV ; thus, precise physical examination in such cases
is needed.

CONFLICT OF INTEREST
The authors have no conflicts of interest to disclose.

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