Original articlePure Membranous Lupus Nephritis: Description of a Cohort of 150 Patients and Review of the LiteratureNefritis lúpica membranosa pura: descripción de una cohorte de 150 pacientes y revisión de la literatura
Introduction
Over 50% of patients with systemic lupus erythematosus (SLE) have clinically significant renal involvement during the course of their disease. Approximately 8–20% of these are found on renal biopsy to have membranous lupus nephropathy (MLN).1, 2 Pure MLN has distinct histologic features compared to proliferative lupus nephritis.3 This difference is reflected in the clinical presentations of proliferative and membranous lupus nephritis, with the former being dominated by a nephritic process whereas MLN is characterised predominantly by nephrotic syndrome.1, 4, 5
Few studies have addressed the course and outcome of MLN directly,4, 6, 7, 8, 9, 10, 11, 12, 13, 14 and their results are contrasting. Most of our knowledge is derived from extrapolations of data of idiopathic membranous nephritis and/or proliferative lupus nephritis. Research on MLN frequency, natural history, prognosis and treatment was further limited due to a lack of uniform definition over the past several decades, making it difficult to get consistent information from the published literature about MLN.1
An Italian study15 has reported the outcome in 103 patients with MLN, but a third of them had mixed forms. More recently, Mejía-Vilet et al.13 have reported the results of a cohort of 60 Hispanic and Mexican-mestizo patients with pure MLN suggesting an impact of ethnicity on the response to different immunosuppressants (azathioprine, cyclophosphamide or mycophenolate).
Large cohorts of patients with pure MLN are lacking, and to date the influence of ethnicity and social conditions on pure MLN outcome has rarely been studied.
The aims of the present study are to evaluate the clinical features, course, outcome and prognostic indicators in pure MLN and to explore the association of ethnicity, socio-economic level, country of residence and the type of health insurance with the course and prognosis of pure MLN.
Section snippets
Patients
This multicentre, retrospective clinicopathologic study evaluated 150 patients with biopsy-proven Class Va pure MLN (modified WHO classification) who underwent renal biopsies between 1978 and 2011 in 24 hospitals in Spain and 1 in the United States. With the aim of gathering a large number of pure MLN patients and taking into account its low prevalence, we chose to include patients diagnosed in a broad temporal range and from different geographical areas. The registers of renal biopsies of each
Comparison Between Both Cohorts
Of the 150 patients included in the analysis, 48 were followed-up in the Hospital for Joint Diseases of New York and 102 in the rheumatology departments of 24 different hospitals in Spain. The clinical characteristics at MLN presentation, administered treatments at any point during the course of the disease and outcome of both cohorts are displayed in Table 1, Table 2, Table 3. Statistically significant differences between both cohorts were found regarding ethnicity distribution, type of health
Discussion
In this study, we retrospectively analysed the baseline and long-term follow-up characteristics of a cohort of 150 patients with pure MLN. Unlike many other studies, we excluded patients with mixed forms of membranous nephritis. There are only a few studies concerning pure MLN, and the conclusions about the presentation and natural course are unclear. This is possibly a consequence of the difficulties to identify histologically pure forms of MLN according to the different classifications and
Conclusion
MLN usually begins with nephrotic syndrome, high proteinuria and normal serum creatinine. Prognosis is favourable in terms of the maintenance of renal function, although proteinuria usually persists over time. Patient and renal survival are high in patients with pure types of MLN but severity at presentation predicts a poor long-term outcome in terms of ESRD. Other factors such as baseline cardiovascular disease and not having a health insurance are also related with poor prognosis.
Protection of human and animal subjects
The authors declare that no experiments were performed on humans or animals for this study.
Confidentiality of data
The authors declare that no patient data appear in this article.
Right to privacy and informed consent
The authors declare that no patient data appear in this article.
Funding
This study had no funding source or sponsor.
Ethics approval: Ethical approval for this study was obtained in February 2011 from the Hospital Universitario Puerta de Hierro Majadahonda of Madrid, Spain.
Conflicts of Interest
The authors declare no conflicts of interest.
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