Efficacy and safety of low-dose corticosteroid with azathioprine in the treatment of elderly-onset IgA vasculitis with nephritis

  1. Hikaru Sugimoto ,
  2. Yasuko Yamaguchi ,
  3. Wako Yumura and
  4. Takashi Takei
  1. Department of Nephrology, Tokyo Metropolitan Geriatric Hospital, Itabashi-ku, Japan
  1. Correspondence to Dr Hikaru Sugimoto; sugimoto.hikaru14@gmail.com

Publication history

Accepted:14 May 2021
First published:02 Jun 2021
Online issue publication:02 Jun 2021

Case reports

Case reports are not necessarily evidence-based in the same way that the other content on BMJ Best Practice is. They should not be relied on to guide clinical practice. Please check the date of publication.

Abstract

The diagnosis of elderly-onset IgA vasculitis (IgAV) and its prognosis can be difficult to ascertain because of its rarity and the frequent presence of comorbidities. Furthermore, the treatment of elderly-onset IgAV remains controversial. We report a case of IgAV in an 87-year-old patient. Renal involvement was detected early during the IgAV follow-up. He was treated with low-dose corticosteroid and azathioprine, which led to a complete remission without any adverse effects. This suggests that precise intervention with early diagnosis and careful renal follow-up may prevent renal failure and that low-dose steroids with azathioprine can be an effective treatment for elderly-onset IgAV with nephritis.

Background

IgA vasculitis (IgAV), also known as Henoch-Schönlein purpura, is a type of small-vessel vasculitis that is common in children. IgAV is a systemic disorder and affects every organ including the skin, joints, kidney, gastrointestinal tract, lungs and central nervous system.1 Common clinical manifestations of IgAV are palpable purpura, arthritis/arthralgias, glomerulonephritis and gastrointestinal symptoms.

The diagnosis of elderly-onset IgAV can be quite difficult because the symptoms vary widely and are relatively common in the elderly, and there are no definitive markers for the diagnosis of IgAV. Some authors have suggested that the low incidence among the elderly can be attributed to either underdiagnosis or misdiagnosis.2 Therefore, careful physical examinations with serum examinations, urinalysis and biopsies are thought to be inevitable. However, some elderly patients have difficulty remaining still during renal biopsies; consequently, renal biopsies can be difficult to perform.3 In such cases, no definitive diagnosis cannot be made.

The treatment of IgAV remains controversial: little data are available regarding the clinical outcomes of large-scale and long-term prospective studies.4 Only a few case series and retrospective studies of elderly-onset IgAV have been reported.5 In some cases, symptomatic treatments are sufficient, since the symptoms are benign and remission can occur spontaneously. Glucocorticoid may be useful to reduce the severity of renal impairment, and the renal prognosis of elderly patients is reportedly favourable when aggressively treated with glucocorticoid.6 However, glucocorticoid cannot prevent renal injury, nor can it reduce recurrences or the long-term progression of nephritis.7–9 Glucocorticoid also has a number of adverse effects, and a consensus regarding its benefit and risk balance has not been reached. In cases of major renal impairment or to suppress glucocorticoid adverse effects, some immunosuppressive agents have been used.10 11 However, definitive evidence and treatment regimens have not been established.12

Here, we report a case of IgAV in a very elderly patient (>80 years old). Renal involvement was detected early during IgAV follow-up. The patient was treated with low-dose corticosteroid and azathioprine, and a complete remission was achieved without obvious adverse effects. The early diagnosis of IgAV combined with careful renal follow-up is important, especially in the elderly, and low-dose steroid combined with azathioprine may be beneficial for the treatment of elderly-onset IgAV with nephritis.

Case presentation

An 87-year-old Japanese man was admitted to our hospital to investigate a 90-day history of palpable purpura on his leg (figure 1). He had also experienced newly developed arthritis. At that time, abdominal symptoms and renal involvement were not observed. He was diagnosed as having IgAV according to the American College of Rheumatology (ACR) classification criteria and the European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) classification criteria, and he was treated with 5 mg/day of prednisolone (PSL) and non-steroidal anti-inflammatory drugs (NSAIDS). One month later, pitting oedema, proteinuria and haematuria began to appear. He was readmitted to our hospital for the investigation and treatment of nephritis. He had a history of hypertension, a femoral neck fracture and tuberculosis. However, he was diagnosed with hypertension just before his admission, he did not take any antihypertensive medications. He did not have any risk factors to IgA nephritis, such as inflammatory bowel disease, liver disease, some infection and ankylosing spondylitis. He had no habit of NSAIDS consume, and no history of smoking. He used a wheel walker.

Figure 1

Bilateral lower palpable purpura.

On the second hospital admission, a physical examination showed pitting oedema and arthritis, but not abdominal pain. His blood pressure was 145/81 mm Hg, his pulse rate was 61 beats per minute and his body temperature was 36.4℃. He was 159.6 cm in height and 58 kg in weight, corresponding to a body mass index of 22.6 kg/m2.

Investigations

The laboratory results showed a white cell count of 11.9× 109/L (neutrophils: 77.3%, lymphocytes: 18.0%, monocytes: 3.3%, neutrophil to lymphocyte ratio (NLR): 4.3), and the number of platelets was not decreased (270× 109/L). His serum albumin level was reduced to 2.7 g/dL, while his serum IgA was increased to 458 mg/dL. His estimated glomerular filtration rate (e-GFR) was 71.7 mL/min/1.73 m2. e-GFR was calculated using the Japanese society method (male e-GFR (mL/min/1.73 m2):=194*Cre−1.094*age−0.287).13 He had haematuria and proteinuria with preserved eGFR; the number of red blood cells was 50–99/high power field, and the total protein/creatinine value was 10.26 g/gCr. Therefore, he was diagnosed with nephrotic-nephritic syndrome. Tests for antinuclear antibody, myeloperoxidase-antineutrophil cytoplasmic antibodies, and proteinase3-antineutrophil cytoplasmic antibodywere all negative. A skin biopsy showed leucocytoclastic vasculitis (figure 2). IgA deposition in skin lesions was negative, but C3 was positive. He had difficulty remaining still during the renal biopsy because of cognitive impairment and did not provide consent for the procedure; therefore, a renal biopsy was not conducted (table 1). He scored 20 on a Mini-Mental State Examination, 11 on a Frontal Assessment Battery and had an IQ of 39 on Koh’s block design test. Malignancies were not detected using CT scans and endoscopy.

Figure 2

The pathological findings of the skin biopsy (upper: ×10, lower: ×60). The arrow indicates leucocytoclastic vasculitis.

Table 1

Clinical and laboratory profile of the patient at the admission

Clinical and laboratory profile
ADL, activities of daily living; ANA, antinuclear antibody; BMI, body mass index; bpm, beats per minute; BUN, blood urea nitrogen; CRP, C reactive protein; e-GFR, estimated glomerular filtration rate; HbA1c, hemoglobin A1c; HCV, hepatitis C virus ; HPF, high power field; MPO-ANCA, myeloperoxidase-antineutrophil cytoplasmic antibodies; PR3-ANCA, proteinase3-antineutrophil cytoplasmic antibody; TP/Cr, total protein/creatinine.
ADL Independent
Current smoking –
BMI (Kg/m2) 22.6
Blood pressure (mm Hg) 145/81
Pulse rate (bpm) 61
Features at IgA vasculitis
 Gastrointestinal symptom –
 Arthritis/arthralgia +
 Oedema +
 Palpable purpura +
Laboratory tests
 Haematuria (/HPF) 50–99
 TP/Cr (g/gCr) 10.26
 Selectivity index 0.25
 Neutrophil to lymphocyte ratio 4.3
 Haemoglobin (g/dL) 11.9
 Platelet (/μL) 270
 D-dimer (μg/mL) 1.7
 Albumin (g/dL) 2.7
 CRP (mg/dL) 1.4
 BUN (mg/dL) 15
 Creatinine (mg/dL) 0.77
 e-GFR (mL/min/1.73 m2) 71.7
 HbA1c (%) 5.9
 IgG (mg/dL) 1971
 IgA (mg/dL) 536
 IgM (mg/dL) 48
 IgE (IU/mL) 190
 CH50 (U/mL) 100.7
 C3 (mg/dL) 130
 C4 (mg/dL) 38
 ANA ＜40
 SS-A/RO antibody –
 MPO-ANCA –
 PR3-ANCA –
 Cryoglobulin –
 HCV antibody –
 Quantiferon –
 XIII (%) 62
Biopsy findings
 Skin Leucocytoclastic vasculitis
 Kidney –

Differential diagnosis

The diagnosis of elderly-onset IgAV can be quite difficult; NSAIDS can relieve joint or abdominal pain, and IgA deposition in skin lesions can be negative14–16 as was seen in this case. Furthermore, IgAV clinical manifestations such as purpura, arthritis, abdominal pain and renal involvement are relatively common in the elderly, while in elderly-onset nephritis, IgAV is less likely to be the cause.17 He was diagnosed as having IgAV according to the ACR classification criteria and the EULAR/PRINTO/PRES classification criteria, and the most probable cause of nephritis was IgAV, however, these data suggested that the definitive cause of nephritis was unclear.

A renal biopsy is important for the diagnosis and the prognosis of IgAV. Although nephrologists are often reluctant to perform renal biopsies in elderly patients, in many cases the benefits overweigh the risks.18 However, some elderly patients, like our case, cannot remain still during a renal biopsy, making the procedure difficult to perform.3 Therefore, we could only guess that he was having IgAV with nephritis from physical and serum examinations, urinalysis, and a skin biopsy.

Treatment

The renal prognosis of elderly-onset IgAV patients is reportedly favourable when patients are treated aggressively with glucocorticoid.6 However, some reports have indicated that glucocorticoid cannot reduce recurrences or the long-term progression of nephritis,7–9 in addition to having a number of adverse effects. The patient had a history of femoral neck fracture and tuberculosis; therefore, we did not choose to treat him aggressively with glucocorticoid. However, since he was an elderly patient with hypoalbuminaemia and was at high risk of renal impairment,6 we decided to perform some kind of therapeutic intervention on him. In cases with major renal impairment or to suppress the adverse effects of glucocorticoid, some immunosuppressive agent including azathioprine has been prescribed.10 For elderly-onset IgAV, a combination of low-dose steroids and an immunosuppressive agent has been reported to be effective11; although there is no definitive diagnosis of IgAV and no established treatments for this kind of cases, we decided that the combination of low-dose steroids and azathioprine should be initiated to avoid deterioration of renal function.

The clinical course of the patient is shown in figure 3. An antihypertensive drug, a calcium antagonist, and heparin were started on admission. He was treated with 25 mg/day of PSL, followed by PSL dose reduction and azathioprine (25 mg/day).

Figure 3

Clinical course of the patient. The dotted line indicates e-GFR, and the solid line indicates urinary total protein/creatinine. AZA, azathioprine; e-GFR, estimated glomerular filtration rate; PSL, prednisolone; U-TP/Cr, urinary total protein/creatinine.

Outcome and follow-up

This treatment enabled a complete remission of the nephritis, and no relevant adverse events occurred (figure 3). Although spontaneous remission cannot be completely ruled out, considering the decrease in proteinuria was observed immediately after the hospitalisation, the use of the antihypertensive drug, dietary therapy such as salt restriction, which reduced blood pressure from 140–160 mm Hg to 120–130 mm Hg, longer time spent in bed, administration of heparin, which has proteinuria-lowering effects, may be the reasons for the decrease in proteinuria, especially in the first 10 days. This is also suggested by the fact that the purpura resolved later than the decrease in proteinuria. His renal function had been stable for more than 1 year, and proteinuria and haematuria had disappeared (table 2). Furthermore, he had been free from arthritis, stomachache and purpura, therefore, the administration of steroid and azathioprine was ended, finally.

Table 2

Clinical and laboratory profile of the patient 1 year after starting the treatment

Clinical and laboratory profile
BUN, blood urea nitrogen; e-GFR, estimated glomerular filtration rate; HPF, high power field; TP/Cr, total protein/creatinine.
Features at IgA vasculitis
 Gastrointestinal symptom –
 Arthritis/arthralgia –
 Oedema –
 Palpable purpura –
Laboratory tests
 Haematuria (/HPF) <1
 TP/Cr (g/gCr) 0
 Albumin (g/dL) 3.7
 BUN (mg/dL) 22
 Creatinine (mg/dL) 0.78
 e-GFR (mL/min/1.73 m2) 70.4

Discussion

Here, we have described a case of IgAV in a very elderly patient (>80 years old). Renal involvement was detected early during IgAV follow-up. The patient was treated with low-dose corticosteroid and azathioprine, and achieved a complete remission without obvious adverse effects. This case highlighted two issues: precise intervention with early diagnosis and careful renal follow-up may prevent renal failure with elderly-onset IgAV, and treatment with azathioprine and low-dose corticosteroid may be useful for elderly-onset IgAV with nephritis.

The diagnosis and prognosis of elderly-onset IgAV can be difficult because IgAV symptoms vary widely and are relatively common in the elderly, there are no definitive diagnostic and prognostic markers of IgAV and the initial presentation is not correlated with the long-term outcome.4 A renal biopsy provides diagnostic and prognostic information regarding renal survival. However, some elderly patients, like our case, cannot remain still during a renal biopsy, making the procedure difficult to perform.3 In addition, patients with low-grade histological lesions may develop chronic renal failure, and patients with higher histological stages may experience spontaneous remission.19 The proportions of crescents are not associated with a higher risk of end-stage renal disease or a 50% decline in renal function after adjusting for potential confounders.20 Several factors, other than renal histopathology, are reportedly related to renal involvement, including onset age, gender, smoking status, distribution of skin lesions, persistent purpura, abdominal pain, severe bowel angina, gastrointestinal bleeding, arthritis/arthralgia, relapse, blood pressure, leucocytosis, thrombocytosis, CRP, ASO, ESR, IgA, IgE, IgG, C3, serum albumin, GFR, nephritic-nephrotic syndrome and the NLR (table 3).6 21–26 These indicate that the patient was at high risk of renal impairment, and we decided to provide some therapeutic intervention on him. Although it is not possible to generalise whether this prognosis and the treatment will be successful in all cases, this case may provide some teaching points for treating similar cases where renal biopsies cannot be conducted. In addition, no definitive predictors exist and corticosteroids are not preventive for any degree of risk7–9 and are not beneficial after the renal function is severely impaired. Therefore, if an elderly person has symptoms suggestive of IgAV, it is important to test renal function frequently with serology and urinalysis to detect renal damage at an early stage.

Table 3

Prognostic factors of renal involvement in IgA vasculitis

Prognostic factors of IgA vasculitis (reference 6, 21–26)
ASO, antistreptolysin O; CRP, C reactive protein; ESR, erythrocyte sedimentation rate; GFR, glomerular filtration rate; ISKDC, International Study of Kidney Disease in Children.
Age >80
Gender Man
Current smoking –
Distribution of skin lesions Lower limb
Persistent purpura –
Abdominal symptoms –
Arthritis +
Relapse –
High blood pressure +
Neutrophil to lymphocyte ratio >3.5
thrombocytosis –
Low serum albumin +
Low GFR –
Nephrotic syndrome +
Elevated CRP +
Elevated ESR +
Elevated IgA +
Elevated IgE –
Elevated IgG –
Decreased C3 –
Positive ASO –
Leucocytosis +
Histopathology (ISKDC) –

Footnotes

  • Contributors HS: wrote the manuscript. WY: provided the histopathological image. YY and TT: completed the literature search and found the relevant articles for the discussion.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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