Lupus nephritis: A simplified approach to diagnosis and treatment in South Africa

Lupus nephritis (LN) occurs in 50%–60% of patients with childhood-onset systemic lupus erythematosus (cSLE), leading to significant morbidity. Timely recognition of renal involvement and appropriate treatment are essential to prevent renal damage. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative aimed to generate diagnostic and management regimens for children and adolescents with rheumatic diseases including cSLE. Here, we provide evidence-based recommendations for diagnosis and treatment of childhood LN. Recommendations were developed using the European League Against Rheumatism standard operating procedures. A European-wide expert committee including paediatric nephrology representation formulated recommendations using a nominal group technique. Six recommendations regarding diagnosis and 20 recommendations covering treatment choices and goals were accepted, including each class of LN, described in the International Society of Nephrology/Renal Pathology Society 2003 classification system. Treatment goal should be complete renal response. Treatment of class I LN should mainly be guided by other symptoms. Class II LN should be treated initially with low-dose prednisone, only adding a disease-modifying antirheumatic drug after 3 months of persistent proteinuria or prednisone dependency. Induction treatment of class III/IV LN should be mycophenolate mofetil (MMF) or intravenous cyclophosphamide combined with corticosteroids; maintenance treatment should be MMF or azathioprine for at least 3 years. In pure class V LN, MMF with low-dose prednisone can be used as induction and MMF as maintenance treatment. The SHARE recommendations for diagnosis and treatment of LN have been generated to support uniform and high-quality care for all children with SLE.

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A pathophysiology-based approach to the diagnosis and treatment of lupus nephritis

Kidney International ◽

10.1016/j.kint.2016.05.017 ◽

2016 ◽

Vol 90 (3) ◽

pp. 493-501 ◽

Cited By ~ 29

Author(s):

Hans-Joachim Anders ◽

Brad Rovin

Keyword(s):

Lupus Nephritis ◽

Diagnosis And Treatment

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Comparing the Efficacy and Safety of Induction Therapies for the Treatment of Patients with Proliferative Lupus Nephritis in South Africa

International Journal of Nephrology ◽

10.1155/2020/2412396 ◽

2020 ◽

Vol 2020 ◽

pp. 1-7

Author(s):

Phelisa Sogayise ◽

Udeme Ekrikpo ◽

Ayanda Gcelu ◽

Bianca Davidson ◽

Nicola Wearne ◽

...

Keyword(s):

South Africa ◽

Lupus Nephritis ◽

Induction Therapy ◽

Randomized Study ◽

Induction Treatment ◽

Limited Data ◽

Proliferative Lupus Nephritis ◽

Significant Difference ◽

Kidney Replacement Therapy

Background. Lupus nephritis (LN) can be complicated with requirement for kidney replacement therapy and death. Efficacy of induction therapies using mycophenolate mofetil (MMF) or intravenous cyclophosphamide (IVCYC) has been reported from studies, but there is limited data in Africans comparing both treatments in patients with proliferative LN. Methods. This was a retrospective study of patients with biopsy-proven proliferative LN diagnosed and treated with either MMF or IVCYC in a single centre in Cape Town, South Africa, over a 5-year period. The primary outcome was attaining complete remission after completion of induction therapy. Results. Of the 84 patients included, mean age was 29.6 ± 10.4 years and there was a female preponderance (88.1%). At baseline, there were significant differences in estimated glomerular filtration rate (eGFR) and presence of glomerular crescents between both groups ( p ≤ 0.05 ). After completion of induction therapy, there was no significant difference in remission status (76.0% versus 87.5%; p = 0.33 ) or relapse status (8.1% versus 10.3%; p = 0.22 ) for the IVCYC and MMF groups, respectively. Mortality rate for the IVCYC group was 5.5 per 10,000 person-days of follow-up compared to 1.5 per 10,000 person-days of follow-up for the MMF group ( p = 0.11 ), and there was no significant difference in infection-related adverse events between both groups. Estimated GFR at baseline was the only predictor of death (OR: 1.0 [0.9–1.0]; p = 0.001 ). Conclusion. This study shows similar outcomes following induction treatment with MMF or IVCYC in patients with biopsy-proven proliferative LN in South Africa. However, a prospective and randomized study is needed to adequately assess these outcomes.

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Diagnosis and Treatment of Lupus Nephritis

Medical Clinics of North America ◽

10.1016/s0025-7125(16)33156-x ◽

1966 ◽

Vol 50 (4) ◽

pp. 1119-1125

Author(s):

Charles D. Farmer ◽

Richard H. Ferguson

Keyword(s):

Lupus Nephritis ◽

Diagnosis And Treatment

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Clinical features and outcome of lupus myocarditis in the Western Cape, South Africa

Lupus ◽

10.1177/0961203316651741 ◽

2016 ◽

Vol 26 (1) ◽

pp. 38-47 ◽

Cited By ~ 11

Author(s):

R Du Toit ◽

P G Herbst ◽

A van Rensburg ◽

L M du Plessis ◽

H Reuter ◽

...

Keyword(s):

South Africa ◽

Lupus Nephritis ◽

Disease Activity ◽

Clinical Features ◽

Wall Motion ◽

Left Ventricular ◽

Western Cape ◽

Laboratory Results ◽

Lupus Myocarditis ◽

Related Mortality

Background African American ethnicity is independently associated with lupus myocarditis compared with other ethnic groups. In the mixed racial population of the Western Cape, South Africa, no data exists on the clinical features/outcome of lupus myocarditis. Objectives The objective of this study was to give a comprehensive description of the clinical features and outcome of acute lupus myocarditis in a mixed racial population. Methods Clinical records (between 2008 and 2014) of adult systemic lupus erythematosus (SLE) patients at a tertiary referral centre were retrospectively screened for a clinical and echocardiographic diagnosis of lupus myocarditis. Clinical features, laboratory results, management and outcome were described. Echocardiographic images stored in a digital archive were reanalysed including global and regional left ventricular function. A poor outcome was defined as lupus myocarditis related mortality or final left ventricular ejection fraction (LVEF) <40%. Results Twenty-eight of 457 lupus patients (6.1%) met inclusion criteria: 92.9% were female and 89.3% were of mixed racial origin. Fifty-three per cent of patients presented within three months after being diagnosed with SLE. Seventy-five per cent had severely active disease (SLE disease activity index ≥ 12) and 67.9% of patients had concomitant lupus nephritis. Laboratory results included: lymphopenia (69%) and an increased aRNP (61.5%). Treatment included corticosteroids (96%) and cyclophosphamide (75%); 14% of patients required additional immunosuppression including rituximab. Diastolic dysfunction and regional wall motion abnormalities occurred in > 90% of patients. LVEF improved from 35% to 47% ( p = 0.023) and wall motion score from 1.88 to 1.5 ( p = 0.017) following treatment. Overall mortality was high (12/28): five patients (17.9%) died due to lupus myocarditis (bimodal pattern). Patients who died of lupus myocarditis had a longer duration of SLE ( p = 0.045) and a lower absolute lymphocyte count ( p = 0.041) at diagnosis. LVEF at diagnosis was lower in patients who died of lupus myocarditis ( p = 0.099) and in those with a persistent LVEF < 40% ( n = 5; p = 0.046). Conclusions This is the largest reported series on lupus myocarditis. The mixed racial population had a similar prevalence, but higher mortality compared with other ethnic groups (internationally published literature). Patients typically presented with high SLE disease activity and the majority had concomitant lupus nephritis. Lymphopenia and low LVEF at presentation were of prognostic significance, associated with lupus myocarditis related mortality or a persistent LVEF < 40%.

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