Further description of early clinically silent lupus nephritis.

Thirty silent lupus nephritis (SLN) patients were compared to 16 individuals bearing overt lupus nephritis (OLN). Results included: years of systemic lupus erythematosus (SLE) diagnosis were significantly earlier (4.6 +/- 2.8 years) in SLN than in OLN (7.18 +/- 3.61) (P < 0.05). Neurological compromise, hypertension, normocitic anemia and lymphopenia were significantly prevalent in OLN than in SLN (P < 0.05). Beside normal urinary sediment and renal function tests, the SLN group showed a moderate increase of both activity (AI) and chronicity (CI) renal pathology index when compared to highly increased AI and CI in OLN (P < 0.05). Seventy percent of SLN patients were ISN/RPS Classes I (6.6%) and II (63.3%) while 81% of OLN cases were Classes III, IV (37.5%) and V. IgG, IgA, IgM, lambda chain, C3 and fibrinogen immune deposits were found in 90% or over in both SLN and OLN individuals while in 60% or over, both groups also showed kappa chain, Clq and C4 deposits. While prevalence of ANA, anti-dsDNA and anti-C1q antibodies were similar in both groups, anti-histone, anti-RNP, CIC and CH50 serum levels were significantly different in OLN versus SLN (P < 0.05). We strongly suggest that indeed SLN is the earliest stage in the natural history of lupus nephritis.

Nefropatía silente en lupus eritematoso sistémico / Silent lupus nephritis

La nefropatía lúpica (NL) incrementa la morbilidad y mortalidad asociada al lupus eritematoso sistémico (LES) pero el compromiso renal se expresa clínicamente, sólo en unas dos terceras partes de los pacientes. Un alto porcentaje de pacientes con LES pueden tener alteraciones morfológicas renales sin manifestaciones clínicas. Esta condición ha sido llamada nefropatía lúpica silente (NLS) y sólo puede ser confirmada por biopsia renal. Recientemente, nosotros detallamos las características inmunoclínicas y patológicas de la NLS en 41 de 42 pacientes con LES sin manifestaciones clínicas renales. La información colectada en este estudio y la obtenida de la búsqueda bibliográfica realizada, conforman la base de este artículo de revisión en el que se analizan las características patogénicas, inmunoclínicas, histopatológicas, de evolución y de pronóstico de esta patología. Independientemente de las controversias relativas al diagnóstico, pronóstico y tratamiento de la NLS, nosotros creemos que se requiere de un diagnóstico histológico preciso para el seguimiento y tratamiento adecuado de la lesión glomerular en NL, incluyendo aquellos pacientes con NLS. Se requieren además estudios prospectivos para la búsqueda de marcadores confiables inmunopatológicos con el fin de precisar no sólo los patrones posibles de progresión de la NLS sino su respuesta a protocolos terapéuticos razonables (AU)

Silent nephritis in systemic lupus erythematosus.

Silent lupus nephritis (SLN) was investigated in 42 renal asymptomatic patients and compared with 49 untreated patients with overt lupus nephropathy (OLN). Urinary sediment, quantitative proteinuria, creatinine clearance, antinuclear antibodies (ANA), complement, circulating immune complexes (CIC) and renal biopsies were evaluated in all of the patients. Forty-one out of the 42 (97.6%) patients had SLN according to histopathological findings. Results showed that the mean age, female/male ratio and the clinical activity index (SLEDAI) were similar in both groups (P > 0.05). The prevalence of ANA, anti-ds DNA, anti-ENA autoantibodies and C4 serum levels showed no statistical differences between the two groups (P > 0.05). Conversely, in the OLN group, elevated CIC and diminished CH50 and C3 serum levels were significantly different (P < 0.01). WHO class II was the predominant renal lesion in the group with SLN (P < 0.0001), whereas class IV was in the OLN patients (P < 0.0001). We conclude that, in our series, SLN was highly prevalent in renal asymptomatic patients with otherwise systemic lupus erythematosus. Furthermore, abnormal levels of CIC, CH50 and C3 associated with WHO class II suggest a moderate but ongoing activation of immune-mediated renal injury mechanisms.

Cluster analysis of antinuclear autoantibodies in the prognosis of SLE nephropathy: are anti-extractable nuclear antibodies protective?

To investigate the possible role of anti-ENA autoantibodies in the pathogenesis of SLE nephropathy, we performed a cross sectional clustering study of 91 SLE patients using 75 clinical and laboratory variables examining the presence of anti-dsDNA and ENA autoantibodies by ELISA and Western blot. We applied principal component, hierarchical cluster, multiple correspondence and logistical regression analysis. Two polar forms of SLE nephropathy and five clinical groups were identified: group 1 without overt nephropathy (n = 37), group 2 with nephropathy and only proteinuria (n = 19), group 3 nephropathy and only hematuria (n = 11), group 4 with hematuria and proteinuria (n = 14) and group 5 on renal failure (n = 10). When analyzed individually, levels of anti-dsDNA and single anti-ENA antibodies did not allow us to differentiate between renal and non-renal groups. However, when the anti-ENA autoantibodies were analyzed as a cluster, a high predictive value for clinical nephropathy was obtained. Thus, the absence of ENA antibodies (ENA ve or Venezuelan cluster) increased eleven-fold the odds ratio to develop SLE nephropathy. We suggested that the ENA ve cluster may predict development of the most severe forms of renal lupus while the ENA Sm/RNP and the ENA Ro/La/Sm/RNP clusters could be associated with the absence and the most benign form of SLE nephropathy. It must be interesting to apply similar cluster methodology in an SLE population with different ethnic background.

Auricular chondritis and diffuse proliferative glomerulonephritis in primary Sjögren's syndrome.

Bilateral auricular inflammation with histological changes of relapsing polychondritis was observed in a female patient with primary Sjögren's syndrome. This was accompanied by rapidly progressive renal insufficiency due to diffuse proliferative glomerulonephritis. To our knowledge this is the first well documented case of primary Sjögren's syndrome associated with chondritis and glomerulonephritis, further emphasising the wide spectrum of extraglandular manifestations in this autoimmune disorder.

Clinical and histological kidney involvement in human kala-azar.

A 19-year-old women developed prolonged fever, weight loss, hepatosplenomegaly, anemia, leukopenia, and hyperglobulinemia. Appropriate tests indicated that she had visceral leishmaniasis (kala-azar). Urinalysis demonstrated significant proteinuria and microhematuria with the presence of red cell casts. A kidney biopsy was performed. Light microscopy showed a slight mesangial thickening and segmental mesangial proliferation. Immunofluorescence demonstrated deposits of immunoglobulins A and M, complement, and fibrinogen. Electron microscopy showed subendothelial and intramembranous deposits. After treatment with N-methylglucamine antimonate the proteinuria and microhematuria disappeared and the patient recovered uneventfully.