Association between the presence of anti-c1q antibodies and active nephritis in patients with systemic lupus erythematosus

Lupus nephritis (LN) is a severe complication of systemic lupus erythematosus (SLE). A retrospective analysis was carried out on a group of 24 patients with SLE to evaluate whether the presence of anti-C1q antibodies (anti-C1q) is related to renal involvement and to explore the behaviour of anti-C1q with respect to LN during a four-year follow-up period. A first serum sample stored at the serum bank, taken not more than three years after SLE diagnosis and one serum sample per year for the subsequent four years were used to detect anti-C1q. Lupus clinical manifestations and serological markers of activity corresponding to the date of each serum sample selected were collected from medical records. In the first serum sample, anti-C1q were found in 8 active SLE. LN was confirmed by histology in 5/8 patients who were positive for anti-C1q and in 1/16 patients who were negative for these autoantibodies (p = 0.0069). Three patients (3/8) had anti-C1q without renal involvement but with lupus skin manifestation. Anti-C1q levels decreased in 3/5 patients with LN who responded to treatment and remained higher in 2/5 patients who needed a new renal biopsy which showed severe renal disease. The 15 patients without severe kidney disease and anti-C1q negative at diagnosis did not develop LN and anti-C1q remained negative in the 4 years of follow up. Anti-C1q were found in SLE patients with active renal involvement or with lupus skin disease. The absence of anti-C1q seemed to be linked to low probabilities of renal involvement.

La nefritis lúpica (NL) es una complicación grave del Lupus Eritematoso Sistémico (LES). Se analizó retrospectivamente en 24 pacientes con LES si la presencia del anticuerpo anti-C1q (anti-C1q) se asociaba con NL y el comportamiento del anti-C1q respecto a la NL en un período de seguimiento de cuatro años. El anti-C1q se determinó en una primera muestra de suero no distante en más de tres años del diagnóstico de LES y en una muestra por año en los siguientes cuatro años. Se obtuvo información de las historias clínicas, sobre manifestaciones clínicas de LES y marcadores serológicos de actividad para las fechas de selección de cada suero. En la primera muestra de suero se detectó anti-C1q en 8 pacientes con LES activo. NL fue confirmada por histología en 5 de ellos y en uno de 16 pacientes con anti-C1q negativos (p = 0.0069); 3 de 8 pacientes fueron anti-C1q positivos sin NL y con lesiones en piel. Los niveles de anti-C1q disminuyeron en 3/5 pacientes con NL que respondieron al tratamiento y se mantuvieron aumentados en 2/5 que necesitaron una nueva biopsia, que evidenció compromiso renal grave. Los 15 pacientes sin enfermedad renal grave y con anti-C1q negativo al diagnóstico no desarrollaron NL y el anti-C1q se mantuvo negativo en los 4 años de seguimiento. El anti-C1q se asoció en pacientes con LES a NL activa o con compromiso en piel. La ausencia del anti-C1q parecería relacionarse a un menor riesgo de desarrollar nefropatía lúpica.

Childhood lupus: Experience from eastern India.

Objective. To delineate the clinical behavior of SLE in children from Eastern India and to the differences in disease pattern. Methods. In the present study, all 44 patients of pediatric SLE who were diagnosed over a period of 5 years in our pediatric rheumatology clinic were followed prospectively. The resultant database was analyzed using standard statistical methods. Results. About 3.9% of all rheumatology cases dealt with in the clinic in the last five yrs (n=1063) were SLE. The number of children in 5-8 yrs and 8-12 yrs age groups were 13 and 27, respectively. The overall female (n=35) to male (n=9) ratio in this study was 3.8:1. Renal, hematological and Neuropsychiatric features were most common major organ manifestations(54%,54% and 25% respectively). Joints and skin were the most common minor organs involved. Two case were ANA negative SLE. Among the typical features of ANA negative disease, only nephritis was found in these patients. Anti dsDNA was positive in 50 % cases (n=21). C3 levels were studied in all cases with nephritis (n=22) and 68 % (n=15) had hypocomplementemia. Anti Ro and anti La antibodies were positive in two cases of neonatal lupus. APLA, Anti Sm antibody and anti U1RNP were negative in the cases where testing was done. Conclusions. This study has tried to delineate the disease trends of childhood lupus from Eastern India. Certain important trends have emerged which are different from other contemporary Indian and International observations.

Lupus nephropathy: clinical, immunohistochemistry and quantitative morphometric analysis studies

Lupus nephritis includes a wide range of parenchymal injuries and severity. Better predictors to outcome are needed for patients newly diagnosed with lupus nephritis, so that an appropriate management strategy may be selected. This study aimed to determine whether the ratio of hepatocyte growth factor [HGF] to transforming growth factor beta 1 [TGF beta1] in lupus nephritis could be a prognostic factor for response to therapy with cyclophosphamide and steroids at six months. Also, to determine whether a simple automated system for objective scoring of biopsies of lupus nephritis could be a prognostic factor for response to therapy with cyclophosphamide and steroids at 6 months. Consequently, renal biopsy findings and clinical parameters of thirty parasites-free patients with new onset lupus nephritis were recorded. Histopathologic, clinical, immune-histochemical and morphometric data at baseline served to define the predictive value for outcome after 6 months of therapy. The results showed a significant positive relationship between response to therapy and HGF IS [P= 0.007], HGF ES [P= 0.026], HGF IS/ TGFbeta1 IS ratio [P= 0.022] and HGF ES/ TGFbeta1 ES ratio [P= 0.001]. A significant inverse relationship was proved between response to therapy and TGFbeta1 IS [P= 0.025] as well as TGFbeta1 ES [P= 0.017]. Also, a significant inverse relationship was present between response to therapy and nuclear index, tubular index and matrix index [P = 0.03, 0.03 and 0.029 respectively]

Clinical features and outcome of systemic lupus erythematosus.

We report the clinical profile, treatment and outcome of systemic lupus erythematosus in 70 patients between the age of 4-15 years. Fever (94.2%), arthritis (65.7%) and malar rash (57.1%) were the chief extra-renal manifestations. The ESR was raised in 98.5% patients, anemia was seen in 60% and direct Coombs test was positive in 58.3%. Antinuclear antibody was positive in all; anti-double stranded DNA antibody and low C3 levels were seen in 77.1% and 80%, respectively. Renal involvement was noted in 77.1% and included proteinuria (53%), hematuria (42.8%), hypertension (18.5%) and elevated serum creatinine (8.6%). Renal histology showed class I nephritis in 3.7%, class II in 44.4%, class III in 4.3%, class IV in 44.4% and class V in 1.8%. On follow up 18.8 months later, 70% patients were in remission, 7.5% had active disease and 7.5% died. The characteristics of childhood lupus erytematosus were similar to those previously reported. The outcome was favorable in most cases.

Thymus and activation-regulated chemokine in children with systemic lupus erythematosus

Systemic Lupus Erythematosus [SLE] is an autoimmune rheumatic disease and glomerulonephritis is a challenging complication of SLE which is more frequent in children. Thymus and activation - regulated chemokine [TARC] is a hemostatic chemokine and TARC production is induced rapidly thus providing an important link between early innate immune responses and adaptive immunity. The aim of the present work was to measure serum TARC in SLE patients and correlate it with disease activity in patients with clinically evident lupus nephritis versus patients without lupus nephritis. This study was conducted on thirty patients [26 females and 4 males] with SLE, regularly attending the Pediatric Allergy and lmmunology Clinic Children's Hospital, Ain Shams University. Their ages ranged between 9-16 years [mean +/- SD = 13.6 +/- 2.68 years]. Lupus patients were categorized into two groups according to lupus nephritis [LN]: Group I a [with LN] and Group I b [without LN]. Results of the previous two groups were compared to a control group comprised of 40 [27 females and 13 males] age and sex matched apparently healthy subjects whose ages ranged between 10 and 16 years [mean + SD= 12.95 +/- 2.68 years]. All participants were subjected to full history taking, thorough clinical examination, urine analysis, assessment of ESR, creatinine, C3, ANA Abs, anti-dsDNA Abs and serum TARC. All SLE patients were seropositive for ANA. Serum TARC levels were statistically highly significantly [p=<0.001] elevated in all lupus patients, in Group Ia, in Group lb all respectively compared to controls and also in Group Ia versus Group lb. As regards indicators of LN, serum TARC showed statistically highly significant [p<0.001] elevated levels in patients with hematuria, edema, hypertension and anti-dsDNA positivity than in those with negative indicators of LN. Serum TARC showed statistically significant [p<0.05] positive correlation with ESR and SLEDAT score in all lupus patients, statistically highly significant [p=<0.001] positive correlation with ESR [in group I a], 24 hrs urinary proteins [in all lupus patients and group I b] and serum creatinine [in all lupus patients, group I a and group I b]. In conclusion, serum TARC levels were statistically significantly higher in all lupus patients versus healthy controls being statistically significantly higher in LN patients with versus lupus patients without nephritis. Also, serum TARC had significant positive correlation to SLEDAI score and ESR indicating its correlation with disease activity

Clinical an immunological factors associated with lupus nephritis in patients from northwestern Colombia / Factores clínicos y epidemiológicos asociados con nefritis lúpica en pacientes del noroccidente colombiano

A cross-sectional and multicenter study was undertaken to analyze the clinical and immunological characteristics at diagnosis associated with nephritis in northwestern Colombian patients with systemic lupus erythematosus (SLE). Thirty nine patients with lupus nephritis were included and were compared to 100 SLE patients without nephritis. A multivariate analysis was performed. The patients who developed nephritis had a higher frequency of oral ulcers (41 percent vs. 21 percent, OR3.1, 95 percent CI: 1.3-7.5 p 0.01) and malar erythema (77 percent vs. 45 percent, OR4.4, 95 percent CI: 1.8-10.8 p0.001). Lupus nephritis was observed in 77 percent of cases during the first year of the disease. The frequency of anti-DNA antibodies was higher in patients with nephritis, however, differences were not statistically significant (83 percent vs 64 percent, OR2.6, 95 percent CI: 1.03-6.41, p0.06). The presence of other autoantibodies (anti-Ro, anti-La, anti-RNP, anti-Sm and anticardiolipin) at diagnosis was similar in both groups. This autoantibody profile remained unchanged throughout the evolution of the disease. Patients with lupus nephritis had a higher prevalence of arterial hypertension (60 percent vs 10 percent, OR13.7, 95 percent IC: 5-37, 0.00001) and hyperlipidemia (30 percent vs 7 percent, OR8.1, 95 percent IC: 2.5-27, p0.0006) at onset. Finally, patients with lupus nephritis required more hospitalizations (1) over the course of disease (89 percent vs 60 percent, OR7.8, 95 percent IC: 2.1-29, p0.002). In conclusion, lupus nephritis appears early during the course of SLE. Malar erythema, oral ulcers, hypertension and hyperlipidemia at onset of disease are associated factors. Lupus nephritis is a major risk factor leading to repeated hospitalizations. This study may help to assist in public health policies in our population in order to improve patient outcomes while simultaneously reducing disease costs.

IgA deficiency: a report of three cases from Thailand.

Selective IgA deficiency has been reported to be the most common primary immunodeficiency disease in Western countries. A markedly lower frequency of this condition has been reported in the Japanese population. While most of the IgA deficient cases are healthy, some patients develop significant recurrent sinopulmonary infections, allergic disorders and autoimmune diseases. Herein, we report three cases of IgA deficiency among Thai patients, all of whom suffered from chronic sinopulmonary infections. Two of the three patients had absolute IgA deficiency while the third had a partial IgA deficiency. The associated conditions found in these three patients were deficiencies of an IgG subclass, allergic rhinitis and lupus nephritis. The youngest child (5 years old boy with lupus nephritis) expired from Pneumocystis carrinii pneumonia complicated with adult respiratory distress syndrome.

Class specific antinuclear antibodies in systemic lupus erythematosus.

The incidence and significance of class specific antinuclear antibodies (ANA), specially with relevance to clinical manifestations of arthritis, renal disease and serositis was studied in 40 patients of systemic lupus erythematosus by the indirect immunofluorescent method. Clinical activity was scored by Morimoto's scoring system. A significant correlation was obtained between IgD ANA and clinical activity of the disease and IgG ANA exhibiting lupus pattern of immunofluorescence and serositis.

Nefritis lúpica I: correlación clínica, inmunológica e histopatológica / Lupus Nephritis I: clinical, inmunologic and histopathological correlation

Con el objeto de establecer correlaciones entre diversos parámetros clínicos y para-clínicos en pacientes con Lupus Eritematoso Sistémico (LES) y Nefropatía Lúpica (NL), se estudiaron 23 pacientes. A todos se le realizó evaluación clínica y paraclínica, y estudio de tejido renal que incluyó contaje de subpoblaciones de células mononucleares (CMo) marcadas con anticuerpos monoclonales (AMo). El estudio inmunopatológico reveló predominio de clase IV (60,87//) OMS de Glomerulonefritis Lúpica (GN-LES). En la mayoría de los casos el tipo y severidad de las lesiones renales no pudo predecirse en base a datos clínicos y de laboratorio. El CH50 y C4 resultaron con niveles séricos más bajos en formas más severas de NL, y se correlacionaron con la actividad clínica, histopatológica y con la reducción de la depuración de creatinina endógena. Todas las clases histológicas de GN-LES mostraron disminución de las células T CD4+ y en la relación CD4/CD8; sólo las clases V y VI tuvieron valores bajos de CD8+. Las mayores poblaciones de CMo del intersticio renal fueron las marcadas con los AMo: HLA-Dr y MQ. La actividad histopatológica se correlacionó con la población MQ+ en intersticio y glomérulo (gl), y con la TAC+ en gl. La hipocomplementemia se correlacionó significativamente con la presencia de células MQ+ tanto en intersticio como en gl. Concluimos, que tanto mecanismos inmunológicos humorales y celulares contribuyen a la patogénesis de la NL

Nefrite lúpica: revisäo de literatura e apresentaçäo de protocolo terapêutico / Lupus nephritis: literature review and report of therapeutic protocol

Os autores revisam conceitos clínicos, histopatológicos e terapêuticos sobre a nefrite lúpica e apresentam seu protocolo

Alteraciones inmunologicas en pacientes con actividad lúpica y compromiso renal / Immunological changes in patients with lupus activity and renal involvement

Una población de pacientes lúpidicos que se encontaban en distintos períodos de actividad y remisión fue evaluadas por parámetros convencionales de laboratorio y por la determinación de anticuerpos anti-ADN fijadores de complemento (anti-ADN FC) en virtud de su posible relación con el compromiso renal y actividad de la enfermedad. Los estudios convencionales de laboratorio confirmaron, como parámetro de elección para determinar el estado de actividad, el nivel de CH50, más aún existiendo compromiso renal. También se observó que los anticuerpos anti-ADN-hemaglutinantes permiten distinguir entre estados de actividad y remisión. Un menor valor discriminativo se adjudicaría a los niveles de complejos inmunes circulantes (CIC) y a los valores de vía alterna del complemento (VAC). Con respecto a los sueros anti-ADN FC positivos se encontró una relación con la actividad lúpica ni con el compromiso renal. Tampoco se observó que los sueros con bajo nivel de CH50 se relacionaran exclusivamente con la presencia de ati-ADN FC; parecería que algún otro mecanismo estaría involucrado en la patogenia de la nefritis lúpica