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IgA vasculitis (Henoch-Schönlein purpura): Kidney manifestations

IgA vasculitis (Henoch-Schönlein purpura): Kidney manifestations
Literature review current through: Jan 2024.
This topic last updated: Jun 21, 2023.

INTRODUCTION — Immunoglobulin A (IgA) vasculitis (IgAV; formerly Henoch-Schönlein purpura [HSP]) is the most common systemic vasculitis among children. Ninety percent of cases occur in the pediatric age group. In contrast to other forms of childhood systemic vasculitis, IgAV is usually self-limited, at least in children, and is characterized by a tetrad of clinical manifestations that vary in their presence and order of presentation:

Palpable purpura (leukocytoclastic vasculitis) in patients with neither thrombocytopenia nor coagulopathy

Arthralgia and/or arthritis

Abdominal pain

Kidney disease

IgAV occurs more often in children than in adults, but kidney involvement is more likely to occur and be more severe in older children and adults, prompting consideration of more aggressive therapy. The kidney manifestations of IgAV, including treatment and prognosis, will be reviewed here. Other aspects of IgAV are discussed separately:

(See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis".)

(See "IgA vasculitis (Henoch-Schönlein purpura): Management".)

EPIDEMIOLOGY AND RISK FACTORS — Kidney involvement has been reported in approximately 20 to 54 percent of children with IgAV [1-7] and may be more prevalent among older children and adults [8].

In an analysis of 161 patients (112 children, 49 adults) with IgAV nephritis from the multicenter Cure Glomerulonephropathy (CureGN) study, the median age at diagnosis was 9 years in children and 35 years in adults [9]. Male sex was predominant among both children and adults with IgAV nephritis (63 and 59 percent, respectively).

Risk factors predicting kidney involvement are not well established, but several clinical and laboratory features have been reported as potential risk factors. In one meta-analysis, older age at onset, gastrointestinal symptoms, persistent purpura, relapse, elevated white blood cell (WBC) count, elevated platelet count, elevated antistreptolysin O (ASLO) titer, and low C3 were associated with kidney involvement in children with IgAV [10]. Other factors associated with the development of kidney disease include delay in diagnosis, angioedema, and central nervous system (CNS) involvement [11,12].

Several reports of IgAV nephritis following coronavirus disease 2019 (COVID-19) or COVID-19 vaccination have been described [13-16]. However, the exact relationship and causality are unclear in most cases.

PATHOGENESIS — IgAV is characterized by the tissue deposition of IgA-containing immune complexes [17]. The histologic findings in the kidney are identical to those in IgA nephropathy, suggesting that the two disorders may have a similar pathogenesis. Likewise, the finding of high circulating levels of galactose-deficient IgA1 (Gd-IgA1) in patients with both IgA nephropathy and IgAV suggests a common underlying pathogenetic mechanism [18]. The observation of the simultaneous occurrence of IgAV and IgA nephropathy in each of two twins after an adenovirus infection is further evidence in support of a common pathogenesis [19]. (See "IgA nephropathy: Pathogenesis".)

IgA deposition is prominent in both IgAV and IgA nephropathy (picture 1), but the kidney injury may be mediated at least in part by immunoglobulin G (IgG) autoantibodies directed against mesangial cell antigens [20,21]. It has also been proposed that IgA1 antibodies generated against endothelial cells could activate neutrophils via the IgA Fc receptor FcalphaRI (CD89), inducing neutrophil migration and activation and ultimately causing tissue damage in IgAV [22]. The course of the kidney disease and circulating antibody titers are roughly parallel, and these autoantibodies do not appear to be present in patients with IgAV who do not have kidney involvement [21].

CLINICAL FEATURES — The classic tetrad of IgAV is rash, arthralgias, abdominal pain, and kidney disease, which can develop in any order and at any time over a period of several days to several weeks [2,23]. (See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis".)

Clinical manifestations — Kidney involvement (IgAV nephritis) typically occurs within a few days to one month after the onset of systemic symptoms [6]. The most common presentation is microscopic or macroscopic glomerular (dysmorphic) hematuria with or without red blood cell (RBC) casts and mild or moderate proteinuria [4,6,9]. Nephrotic-range proteinuria, an elevated serum creatinine, and/or hypertension are present in a minority of patients; these findings are associated with a worse kidney prognosis [6]. (See 'Prognosis' below.)

IgAV nephritis is generally mild in children (particularly young children), while adults are more likely to develop moderate to severe disease, as manifested by a higher frequency of nephrotic syndrome, hypertension, and an elevated serum creatinine [23-27]. In the Cure Glomerulonephropathy (CureGN) cohort of 161 patients with IgAV nephritis, the median estimated glomerular filtration rate (eGFR) at the time of kidney biopsy was 109 mL/min/1.73 m2 among children compared with 67 mL/min/1.73 m2 among adults; only 4 percent of children, compared with 20 percent of adults, had an eGFR <30 mL/min/1.732 [9]. Long-term kidney impairment can occur in 20 to 44 percent of patients who initially present with nephritic or nephrotic syndrome [6,28-30].

There is a general correlation between the severity of the kidney manifestations and the findings on kidney biopsy [28,31-35]. Patients with only asymptomatic hematuria, for example, usually have only focal mesangial proliferation, whereas patients with proteinuria have more marked cellular proliferation and, if nephrotic or nephritic, may have crescent formation [35,36]. Patients with repeated attacks of purpura or macroscopic hematuria often have exacerbation of kidney manifestations and biopsy-confirmed worsening of glomerular lesions. (See 'Pathologic findings' below.)

Pathologic findings — On kidney biopsy, light microscopy can show a wide spectrum of glomerular changes, ranging from isolated mesangial proliferation (picture 2) to focal and segmental proliferation to severe crescentic glomerulonephritis. The diagnostic finding is dominant or codominant IgA deposition, mostly IgA1, in the mesangium on immunofluorescence, similar to that seen in IgA nephropathy (picture 1). In addition, immunofluorescence frequently reveals IgG, immunoglobulin M (IgM), fibrinogen, and C3 in the glomeruli. Immunofluorescence staining for C1q is rarely found in IgAV nephritis and should suggest the possibility of lupus nephritis. By electron microscopy, electron-dense deposits are typically found in the mesangial areas, occasionally extending out into the peripheral capillary loops (picture 3).

EVALUATION AND DIAGNOSIS

Monitoring for kidney disease — Patients with IgAV who do not present with kidney involvement should be monitored for the development of IgAV nephritis:

Children with an initially normal urinalysis should have follow-up urine dipstick testing at least once a week for one month and then once every two weeks for five months since 97 percent of children with IgAV who develop kidney disease do so within this time period [6]. Children with isolated hematuria with nonnephrotic-range proteinuria that persists after six months should have periodic monitoring of the serum creatinine every three to six months as long as the hematuria and proteinuria persist. Children with isolated hematuria without proteinuria do not need periodic monitoring of the serum creatinine.

Adults who present with features of IgAV but without kidney involvement should be monitored with a urinalysis to screen for proteinuria and/or hematuria, serum creatinine, and blood pressure check at three-month intervals for at least one year.

Metabolomic profiling has identified putative biomarkers that may predict the development of kidney disease among patients with IgAV who do not present with kidney involvement [37]; however, additional studies validating these findings in larger populations are needed.

Establishing the diagnosis — The diagnosis of IgAV nephritis is typically based upon the clinical presentation. In most cases in children, the development of hematuria, proteinuria, and/or elevated serum creatinine in association with palpable purpura, abdominal pain, and arthritis/arthralgia is so characteristic that no further investigation is usually needed to confirm the diagnosis. If further confirmation is required, a skin biopsy can be performed. Biopsy of the skin lesions reveals inflammation of the small blood vessels (leukocytoclastic vasculitis), which is most prominent in the postcapillary venules, and IgA deposition by immunofluorescence microscopy (image 1A-B). (See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis", section on 'Diagnosis'.)

A kidney biopsy can also be performed to establish the diagnosis, but this invasive procedure is generally reserved for patients in whom the diagnosis is uncertain or who have more severe kidney involvement. In general, we perform a kidney biopsy in all patients with suspected or confirmed IgAV nephritis who present with proteinuria >1 g/day and/or impaired kidney function to assess the severity of the histologic lesions (particularly the degree of crescent formation), which may guide treatment decisions as well as inform prognosis. This approach is largely consistent with the 2021 Kidney Disease: Improving Global Outcomes (KDIGO) guidelines [38]. Based upon data in patients with IgA nephropathy [39,40], some adult nephrologists choose to biopsy patients with lower levels of proteinuria (eg, >500 mg/day or >750 mg/day). (See 'Pathologic findings' above and 'Prognosis' below.)

Differential diagnosis — The classic tetrad (palpable purpura without thrombocytopenia or coagulopathy, arthritis/arthralgia, abdominal pain, and kidney disease) is virtually pathognomonic of IgAV in children. The differential diagnosis includes antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, clotting disorders (such as the antiphospholipid syndrome), and sepsis [41]. (See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis", section on 'Clinical manifestations in children' and "Clinical manifestations of antiphospholipid syndrome".)

IgAV in adults must be distinguished from other systemic autoimmune diseases (such as hypersensitivity vasculitis, cryoglobulinemia, ANCA-associated vasculitis, and systemic lupus erythematosus) and infection-related glomerulonephritis, which can produce similar symptoms but are treated differently. A period of follow-up may be necessary to distinguish among these conditions. One report of 178 patients with IgAV or hypersensitivity vasculitis found that hypersensitivity vasculitis was present with 74 percent accuracy in patients who had no more than two of the following criteria [42]:

Palpable purpura

Bowel angina

Gastrointestinal bleeding

Hematuria

Age at onset ≤20 years

The combination of kidney disease and hemoptysis can simulate the presentation of ANCA-associated vasculitis (microscopic polyangiitis or granulomatosis with polyangiitis) or anti-glomerular basement membrane (GBM) disease. (See "Glomerular disease: Evaluation and differential diagnosis in adults", section on 'Glomerulonephritis (hematuria with proteinuria, kidney function impairment, or other manifestations)'.)

PREVENTION OF IGAV NEPHRITIS — In patients with IgAV who do not have kidney involvement at the time of presentation, we do not recommend treatment with glucocorticoids to prevent the development of IgAV nephritis.

Early administration (at the time of presentation) of oral glucocorticoids has not been shown to be effective at preventing IgAV nephritis. In one trial, 352 children (age <18 years) with new-onset IgAV were randomly assigned to prednisolone (2 mg/kg/day [maximum dose of 80 mg] for seven days, followed by 1 mg/kg/day [maximum dose of 40 mg] for seven days) or placebo for 14 days [43]. At 12 months after disease onset, the prevalence of proteinuria (defined as a urine protein-to-creatinine ratio >20 mg/mmol) was similar between the two groups; changes in kidney function were not assessed. Similarly, two smaller randomized trials that compared oral prednisone with placebo among children presenting with IgAV found no difference in the risk of kidney involvement (defined by the presence of microscopic hematuria or proteinuria) within one year of disease onset [44,45]. There are no studies evaluating preventive strategies in adults with IgAV.

MANAGEMENT OF IGAV NEPHRITIS — Most patients with IgAV receive only supportive therapy with hydration, rest, and analgesics. Some evidence suggests that glucocorticoids enhance the rate of resolution of the arthritis and abdominal pain; however, they do not appear to prevent recurrent disease [2]. The general approach to therapy of IgAV is discussed elsewhere. (See "IgA vasculitis (Henoch-Schönlein purpura): Management".)

Treatment of IgAV nephritis depends upon whether the patient is a child or an adult and upon the severity of the kidney disease. Immunosuppressive therapy should be considered only in patients who present with marked proteinuria (>1 g/day) and/or impaired kidney function during the acute episode. As previously stated, such patients should undergo a kidney biopsy since the severity of the histologic lesions (particularly the degree of crescent formation) appears to be the best indicator of prognosis. (See 'Establishing the diagnosis' above.)

By contrast, patients with limited evidence of kidney involvement (such as microscopic or macroscopic hematuria with mild proteinuria [<1 g/day] and normal kidney function) are generally not subjected to kidney biopsy and are not given specific therapy but should be followed closely for worsening of proteinuria or impairment of kidney function. However, as previously mentioned, some adult nephrologists choose to biopsy patients with lower levels of proteinuria (eg, >500 mg/day or >750 mg/day) and use the biopsy results to guide treatment for those with evidence of kidney damage. (See 'Establishing the diagnosis' above.)

Initial approach

Children — In children with IgAV nephritis, our approach to treatment depends upon the severity of kidney disease at the time of presentation:

In children who present with limited evidence of kidney involvement (ie, microscopic or macroscopic hematuria, proteinuria <1 g/day, and normal serum creatinine), we do not treat with immunosuppressive therapy for IgAV nephritis. We do not routinely initiate an ACE inhibitor or ARB, since such patients typically fully recover without these agents. We monitor urine protein excretion (by spot urine protein-to-creatinine ratio or 24-hour urine protein collection) once per week for one month, then every two weeks for two months to assess for disease progression. In patients who develop an increase in proteinuria to ≥1 g/day, we perform a kidney biopsy to evaluate the need for more aggressive therapy.

In children who present with more severe kidney involvement (ie, proteinuria ≥1 g/day, elevated serum creatinine, or evidence of crescentic glomerulonephritis [>10 percent crescents] on kidney biopsy), we suggest immunosuppressive therapy with glucocorticoids. We administer three pulses of intravenous (IV) methylprednisolone 1 g/1.73 m2 (one dose daily, or on alternate days, for three doses), followed by oral prednisone 30 mg/m2 (once daily for one month, then every other day for two months). We monitor urine protein excretion (by spot urine protein-to-creatinine ratio or 24-hour urine protein collection) and serum creatinine once per week for one month, then every two weeks for two months. Upon completion of three months of glucocorticoid therapy, subsequent treatment depends upon the degree of persistent proteinuria and kidney function impairment:

In patients who respond to treatment with a reduction in proteinuria to <0.5 g/day and stable or improved serum creatinine, we discontinue glucocorticoids. We continue to monitor urine protein excretion on a monthly basis without additional immunosuppressive treatment.

In patients with persistent proteinuria >0.5 g/day or worsening serum creatinine, we obtain a repeat kidney biopsy to determine if there is ongoing inflammatory injury. We start all patients with persistent proteinuria >0.5 g/day after three months on an ACE inhibitor or ARB [38]. If the kidney biopsy demonstrates evidence of persistent, active inflammation (ie, proliferative glomerulonephritis), we repeat a course of pulse IV methylprednisolone followed by oral prednisone, as detailed above. If the kidney biopsy reveals chronic lesions without active inflammation, we discontinue glucocorticoids and continue the ACE inhibitor or ARB indefinitely to reduce proteinuria, unless contraindicated.

Children who do not respond to glucocorticoids may be candidates for other immunosuppressive therapies. These therapies, and the evidence supporting them, are discussed elsewhere in this topic. (See 'Alternative therapies' below.)

Our approach to therapy in children is largely consistent with the 2021 Kidney Disease: Improving Global Outcomes (KDIGO) clinical practice guidelines [38] and the European consensus-based recommendations for diagnosis and treatment of IgAV [46].

There are no randomized, controlled trials to support the use of glucocorticoids in children with IgAV nephritis, and data come primarily from observational studies. High-dose IV methylprednisolone (250 to 1000 mg/day for three days) followed by oral prednisone (1 mg/kg/day for three months) may be beneficial in patients with severe disease (usually defined as crescentic nephritis) [33,47,48]. This regimen is primarily aimed at reversing the inflammatory process (such as macrophage infiltration), rather than the IgA deposition itself. One prospective, uncontrolled study used this regimen in 38 children presenting with the nephrotic syndrome and/or crescents affecting more than 50 percent of glomeruli [33]. Only four (10 percent) progressed to end-stage kidney disease (ESKD), three of whom had been treated late in the course of their disease. Thus, early therapy may be important in preventing irreversible glomerular injury.

Other regimens that have been evaluated in children with crescentic nephritis include glucocorticoids and azathioprine (19 of 21 children showed improvement in kidney function in an uncontrolled study) [49] and multidrug regimens such as glucocorticoids, cyclophosphamide, and dipyridamole, or glucocorticoids, cyclophosphamide, heparin/warfarin, and dipyridamole [50-53]. However, since spontaneous recovery is often observed in patients with crescent formation, it remains unknown whether these regimens are superior to no or less aggressive therapy.

Adults — In adults with IgAV nephritis, our approach to therapy depends upon the severity of kidney disease at the time of presentation and the likelihood of progression to kidney failure. There are limited data to guide the optimal treatment of adults with IgAV nephritis, and our approach is primarily based upon clinical trials of patients treated for IgA nephropathy (see "IgA nephropathy: Treatment and prognosis"):

In adults who present with limited evidence of kidney involvement (ie, microscopic or macroscopic hematuria, proteinuria <1 g/day, and normal serum creatinine), we do not treat with immunosuppressive therapy for IgAV nephritis. We treat all adults with proteinuria >0.5 g/day with an ACE inhibitor or ARB to reduce proteinuria, unless contraindicated. We monitor urine protein excretion (by spot urine protein-to-creatinine ratio or 24-hour urine protein collection) and serum creatinine every two weeks for one month, then every two months for six months to assess for disease progression. In patients who develop an increase in proteinuria to ≥1 g/day or a sustained increase in serum creatinine greater than that expected from use of an ACE inhibitor or ARB, we perform a kidney biopsy to evaluate the need for more aggressive therapy.

In adults who present with more severe kidney involvement (ie, proteinuria ≥1 g/day, elevated serum creatinine, or evidence of crescentic glomerulonephritis on kidney biopsy), we suggest immunosuppressive therapy with glucocorticoids. We typically administer a six-month course of glucocorticoids similar to that used for patients with IgA nephropathy. We give pulse IV methylprednisolone (250 to 500 mg daily for three days), followed by oral prednisone (0.5 mg/kg orally once daily [maximum dose 40 mg/day]). In addition, we treat all such patients with an ACE inhibitor or ARB to reduce proteinuria, unless contraindicated. We monitor urine protein excretion (by spot urine protein-to-creatinine ratio or 24-hour urine protein collection) and serum creatinine every two weeks for one month and then monthly for the first six months:

In patients who respond to treatment with a reduction in proteinuria to <1 g/day and stable or improved serum creatinine, we gradually taper the prednisone dose. We start the taper at two months of treatment with the plan to decrease the dose by 10 mg every two weeks until the patient reaches a dose of 10 mg daily, at which time treatment can be slowly tapered further. This approach is similar to that used in the treatment of some patients with IgA nephropathy who receive glucocorticoid therapy. (See "IgA nephropathy: Treatment and prognosis".)

In patients who have persistent proteinuria ≥1 g/day or worsening serum creatinine after four to six months of treatment, we obtain a repeat kidney biopsy to determine if there is ongoing inflammatory injury [38]. If the kidney biopsy reveals chronic lesions without active inflammation, we discontinue glucocorticoids and continue treatment with an ACE inhibitor or ARB indefinitely to reduce proteinuria, unless contraindicated. If the kidney biopsy demonstrates evidence of persistent, active inflammation (ie, proliferative glomerulonephritis), either mycophenolate or cyclophosphamide is a reasonable choice for second-line therapy. The evidence for either therapy is based on very limited, low-quality data. Given the potential toxicities of each therapy, we prefer a six-month trial of mycophenolate mofetil (MMF) at 500 to 1000 mg twice daily.

In adults who have a large number of active crescents on kidney biopsy (over 20 to 25 percent), some clinicians would treat with cyclophosphamide, rituximab, or MMF in addition to the six-month course of glucocorticoids. Some contributors to this topic prefer to give cyclophosphamide (500 mg IV every two weeks for a total of six doses), based upon its effectiveness in the treatment of other forms of rapidly progressive crescentic glomerulonephritis, while other contributors prefer to give rituximab. Evidence for these therapies is discussed elsewhere in this topic. (See 'Alternative therapies' below.)

Adults who cannot receive or who do not respond to glucocorticoids may be candidates for other immunosuppressive therapies. These therapies, and the evidence supporting them, are discussed elsewhere in this topic. (See 'Alternative therapies' below.)

Alternative therapies — Although not supported by controlled clinical trials, several alternative therapies have been used with variable efficacy in patients with IgAV nephritis. We reserve the use of these therapies for patients in whom initial therapy as discussed above has not been effective. These therapies, and the evidence supporting their use, are discussed below:

Mycophenolate mofetil – One study compared the effects of MMF plus low-dose prednisone (0.4 to 0.5 mg/kg) with full-dose prednisone (0.8 to 1.0 mg/kg) in 53 patients, aged 14 to 62 years, with IgAV nephritis and heavy proteinuria (>2 g/day) [54]. There was no significant difference in the rate of remission between the treatment groups at 6 and 28 months, suggesting that MMF may be useful for inducing and maintaining remission as a glucocorticoid-sparing agent. In another study of 18 children with IgAV nephritis and nephrotic-range proteinuria who had an inadequate response to glucocorticoids and were treated with MMF, 89 percent showed a significant decrease in proteinuria after one month of treatment [55].

RituximabRituximab has been shown to be effective in the treatment of adults and children with glucocorticoid-resistant IgAV nephritis in a small number of observational studies and case series [56-59]. As an example, in one multicenter study of 22 adults with IgAV, 18 of whom had kidney involvement, patients received rituximab for refractory or relapsing disease or because of contraindications to conventional immunosuppressive therapy [59]. Rituximab was administered either as 375 mg/m2 weekly for four consecutive weeks or as two 1000 mg doses given two weeks apart, alone or in combination with other immunosuppressive therapies. During a median of 24 months, 20 patients (91 percent) achieved remission; 7 of these 20 experienced subsequent relapse of disease. Proteinuria decreased significantly compared with baseline, and estimated glomerular filtration rate (eGFR) remained stable.

Cyclosporine – Limited observational data suggest that cyclosporine may be beneficial in patients with IgAV and severe proteinuria [60,61]:

In one small trial of 24 children with IgAV and nephrotic-range proteinuria or crescentic glomerulonephritis, 15 were randomly assigned to either cyclosporine (5 mg/kg for 12 months) or methylprednisolone (three monthly pulses) followed by oral prednisone for four months; the other nine patients received identical treatment without randomization [62]. All cyclosporine-treated patients achieved resolution of nephrotic-range proteinuria within three months; proteinuria improved in only 6 of 13 patients treated with glucocorticoids. These findings were supported by those of a subsequent nationwide observational study from the same country (Finland) [47].

One study evaluated long-term outcomes of 62 children with IgAV (60 with nephrotic-range proteinuria) who received methylprednisolone pulses followed by oral prednisone (n = 42) or cyclosporine (n = 20) as initial therapy [47]. With a mean follow-up of 10.8 years, mean eGFR at the end of follow-up was similar between the two groups (108 versus 109 mL/min/1.73 m2). Among cyclosporine-treated patients, one (5 percent) had mildly decreased kidney function (eGFR 60 to 89 mL/min/1.73 m2), two (11 percent) had persistent proteinuria, and three (17 percent) had persistent hematuria at last follow-up.

CyclophosphamideCyclophosphamide, alone or with glucocorticoids, does not appear to reduce protein excretion or improve or preserve kidney function in patients with IgAV nephritis [52,63].

IV immune globulin – IV immune globulin (IVIG) has been tried successfully in a small number of patients with IgA nephropathy or IgAV nephritis with heavy proteinuria and a progressive decline in glomerular filtration rate (GFR) [64]. More data are required to confirm benefit. (See "IgA nephropathy: Treatment and prognosis".)

Plasmapheresis – Plasmapheresis has also been used in a number of patients with severe, usually crescentic, disease and rapidly progressive kidney failure [48,65,66]. Its efficacy is uncertain (due, in part, to concurrent administration of glucocorticoids), and there are potential side effects.

PROGNOSIS — Short-term kidney outcomes in IgAV are favorable in most patients, with complete recovery reported in 94 and 89 percent of children and adults, respectively, at a mean of approximately 18 months [23]. The long-term kidney prognosis of IgAV nephritis has, in most studies, been worse in adults than in children, a finding that may be due in part to concurrent chronic kidney disease (CKD) or a longer duration between disease onset and clinical presentation [23].

Among children, the manifestations of active IgAV usually resolve spontaneously. The kidney prognosis is excellent when transient hematuria and proteinuria resolve within several months, a course that is generally associated with only focal glomerular involvement [3,28,32]. Recurrence (relapse) of IgAV is common, occurring in up to one-third of patients, and may be more likely among children with kidney involvement [2,3,67,68]. Recurrent symptoms and signs, which tend to mimic the original episode (but tend to be less severe), are normally observed within four months of resolution of the initial symptoms [2]. Recurrent disease may not predict worse long-term outcomes.

Clinical risk factors for CKD were evaluated in a meta-analysis of nine case-control studies involving 969 patients with IgAV nephritis, which found that older age at disease onset, low glomerular filtration rate (GFR), and an initial presentation with nephrotic syndrome or with nephritic-nephrotic syndrome were associated with a unfavorable kidney outcomes [69]. By contrast, an initial presentation with hematuria and mild proteinuria with or without hematuria was associated with more favorable outcomes.

In addition to these clinical predictors of prognosis, there are also pathologic predictors on kidney biopsy. In general, the extent of kidney injury parallels the clinical severity of kidney disease. The percentage of glomeruli showing crescents seems to be the most important prognostic finding. The following observations were noted in a study of 151 children referred to a major academic center and then followed for 1 to 18 years [36]:

Among patients with crescents involving more than 50 percent of glomeruli, 37 progressed to end-stage kidney disease (ESKD), and another 18 percent had CKD.

Among patients who developed ESKD, 86 percent had crescents affecting more than 50 percent of glomeruli.

Few or no crescents were seen on initial biopsy in the 70 percent of patients who recovered or had only minimal urinary abnormalities at latest examination.

In another study that evaluated histologic predictors of outcome among 159 children with IgAV nephritis, chronic glomerular (glomerulosclerosis) and tubulointerstitial lesions, but not acute glomerular (mesangial proliferation, endocapillary proliferation, crescents) and tubulointerstitial lesions, were associated with a poor kidney prognosis [70].

The Oxford Classification of IgA nephropathy, which has been shown to predict outcomes among patients with IgA nephropathy, has not been validated for adult patients with IgAV nephritis. Two small studies found that endocapillary proliferation on kidney biopsy (E1 lesion) was associated with shorter kidney survival; however, other Oxford Classification lesions were not similarly predictive [71,72]. It remains unclear whether these findings can be applied to larger populations. (See "IgA nephropathy: Treatment and prognosis".)

Persistent and progressive disease — A minority of affected patients have persistent kidney manifestations. This was illustrated in a population-based retrospective study of 69 Spanish children followed for a median of seven years; 12 percent had continued hematuria and proteinuria, which was strongly associated with the presence of nephrotic syndrome at diagnosis (odds ratio 23), but none had kidney function impairment [73].

Late deterioration can occur even among patients with initial complete resolution of kidney manifestations. This issue was addressed in a series of 78 children followed for 23 years; 17 had worsening kidney function, including seven with initial complete kidney function recovery [28]. Furthermore, 16 of 44 pregnancies among previously affected individuals were complicated by hypertension and/or proteinuria even in the absence of active kidney disease.

Some children progress to ESKD and require dialysis, as illustrated in the following reports [26,28,29,32,33,36,74-76]:

Among 88 children followed for a mean of nine years, most (86 percent) had no urinary abnormalities; four had heavy proteinuria, and eight had kidney impairment, of whom three were on dialysis (3 percent) [32].

In a different study, among 83 children followed for a mean of seven years, 7 percent became dialysis dependent, and 14 and 16 percent had a doubling of serum creatinine and persistent proteinuria, respectively [74].

Among adults, reported rates of ESKD are higher than in children, ranging from 10 to 30 percent at 15 years [24,26,74,77-79].

As with other disorders, a poor kidney prognosis is more common among those with the following disease or biopsy manifestations [31,32,36,74,77,80]:

Nephrotic syndrome

Kidney impairment

Hypertension

Crescentic glomerulonephritis (>50 percent)

Tubulointerstitial fibrosis

Late progression may be related to persistent, active disease, as manifested by repeated episodes of macroscopic hematuria, or to irreversible nephron loss during the acute episode, leading to compensatory hyperfiltration (driven in part by intraglomerular hypertension) and hypertrophy in the remaining glomeruli. Over a period of years, these adaptive changes induce further glomerular injury that is independent of the primary disease. (See "Secondary factors and progression of chronic kidney disease".)

KIDNEY TRANSPLANTATION — Kidney transplantation can be performed in patients with IgAV nephritis who progress to end-stage kidney disease (ESKD), although recurrent disease can occur [81-85]. Deposition of IgA in the graft is common, but many cases are subclinical [86]. An early study suggested that clinically relevant recurrence occurs in approximately 35 percent of patients at five years with a rate of graft loss due to recurrent disease of 11 percent [81]. Other reviews suggest a lower rate of recurrence (2.5 and 11.5 percent at 5 and 10 years in one small series) but still show a relatively high risk of graft loss due to recurrent disease [84,87]. Overall graft survival, however, appears to be the same for patients with IgAV compared with other kidney allograft recipients. (See "IgA nephropathy: Recurrence after transplantation", section on 'IgA vasculitis (Henoch-Schönlein purpura)'.)

The diagnosis of recurrent kidney disease is based not only upon the demonstration of mesangial IgA deposits but also clinical features of the disease, since isolated deposits can be seen without any clinical features of IgAV. It is possible that asymptomatic IgA deposits may have been present in the donor kidney prior to transplantation. Several reports have documented this phenomenon, but the IgA deposits disappeared within weeks after transplantation due, presumably, to the lack of circulating IgA-containing immune complexes in the recipient [88,89]. Thus, persistent deposits presumably reflect recurrent disease.

Recurrent glomerular disease, often in association with active extrarenal involvement, can lead to loss of the graft [81,82,84,90]. Although one study suggested that this was more likely in patients with aggressive initial disease and relatively rapid progression to ESKD [81], the association between recurrence and severity of the original disease was not confirmed in a later report [84]. It is recommended by some that transplantation be delayed for 12 to 24 months after the disappearance of purpura [81-83]. However, this approach does not prevent recurrent disease in all patients [81].

Some observations suggest that the risk of recurrent disease may be higher in recipients of living, related-donor transplants, a finding similar to that seen in IgA nephropathy [83,84]. However, one retrospective series of 339 patients reported similar rates of recurrence for those who received kidneys from deceased donors versus living, related donors (13 versus 14.3, respectively) [87].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Glomerular disease in adults".)

SUMMARY AND RECOMMENDATIONS

Pathogenesis – Immunoglobulin A (IgA) vasculitis (IgAV) is characterized by the tissue deposition of IgA-containing immune complexes. The histologic findings in the kidney and presence of galactose-deficient IgA1 (Gd-IgA1) in the circulation are identical to those in IgA nephropathy, suggesting that the two disorders may have a similar pathogenesis. (See 'Pathogenesis' above.)

Clinical manifestations Kidney involvement (IgAV nephritis) typically occurs within a few days to one month after the onset of systemic symptoms. The most common presentation is microscopic or macroscopic glomerular (dysmorphic) hematuria with or without red blood cell casts and mild or moderate proteinuria. Nephrotic-range proteinuria, an elevated serum creatinine, and/or hypertension are present in a minority of patients; these findings are associated with a worse kidney prognosis. IgAV nephritis is generally mild in children, while adults are more likely to develop moderate to severe disease. (See 'Clinical manifestations' above.)

Diagnosis – The diagnosis of IgAV nephritis is typically based upon the clinical presentation. If further confirmation is required, a skin biopsy can be performed. A kidney biopsy is generally reserved for patients in whom the diagnosis is uncertain or who have more severe kidney involvement. (See 'Establishing the diagnosis' above.)

Prevention – In patients with IgAV who do not have kidney involvement at the time of presentation, we do not treat with glucocorticoids to prevent the development of IgAV nephritis. (See 'Prevention of IgAV nephritis' above.)

Treatment – Treatment of IgAV nephritis depends upon whether the patient is a child or an adult and upon the severity of the kidney disease:

Children – In children with IgAV nephritis, our initial approach to treatment is as follows:

-Children who present with limited evidence of kidney involvement (ie, microscopic or macroscopic hematuria, proteinuria <1 g/day, and normal serum creatinine) do not require treatment with immunosuppressive therapy for IgAV nephritis. We do not routinely initiate an angiotensin-converting enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB), since such patients typically fully recover without these agents. We monitor urine protein excretion once per week for one month, then every two weeks for two months to assess for disease progression. In patients who develop an increase in proteinuria to ≥1 g/day, we perform a kidney biopsy to evaluate the need for more aggressive therapy.

-For children who present with more severe kidney involvement (ie, proteinuria ≥1 g/day, elevated serum creatinine, or evidence of crescentic glomerulonephritis on kidney biopsy), we suggest immunosuppressive therapy with glucocorticoids rather than no immunosuppression or other immunosuppressive agents (Grade 2C). (See 'Children' above.)

Adults – In adults with IgAV nephritis, our initial approach to treatment is as follows:

-Adults who present with limited evidence of kidney involvement (ie, microscopic or macroscopic hematuria, proteinuria <1 g/day, and normal serum creatinine) do not require treatment with immunosuppressive therapy for IgAV nephritis. We treat all adults with proteinuria >0.5 g/day with an ACE inhibitor or ARB to reduce proteinuria, unless contraindicated. In patients who develop an increase in proteinuria to ≥1 g/day or a sustained increase in serum creatinine greater than that expected from use of an ACE inhibitor or ARB, we perform a kidney biopsy to evaluate the need for more aggressive therapy.

-For adults who present with more severe kidney involvement (ie, proteinuria ≥1 g/day, elevated serum creatinine, or evidence of crescentic glomerulonephritis on kidney biopsy), we suggest immunosuppressive therapy with glucocorticoids rather than no immunosuppression or other immunosuppressive agents (Grade 2C). In addition, we treat all such patients with an ACE inhibitor or ARB to reduce proteinuria, unless contraindicated.

-In adults who have a large number of active crescents on kidney biopsy (over 20 to 25 percent), some clinicians would treat with cyclophosphamide, mycophenolate mofetil (MMF), or rituximab in addition to the six-month course of glucocorticoids. (See 'Adults' above.)

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Topic 3107 Version 28.0

References

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