RESUMEN

Rheumatoid arthritis (RA) is one of the most prevalent inflammatory rheumatic diseases. As it is a chronic and a lifelong destructive disease, the aim of the treatment is to reduce disability and improve quality of life. The Rheumatoid Arthritis Quality of Life (RAQoL) questionnaire is a patient-reported outcome measure, specific to RA. To adapt and validate the RAQoL for use in Serbia, two translation panels were involved to produce the Serbian RAQoL. After successful translation, face and content validity was determined via cognitive debriefing interviews. The psychometric properties of the questionnaire were examined, including reliability and construct validity, by using the Nottingham Health Profile (NHP) as a comparator scale. The RAQoL was translated successfully and rated as applicable, relevant and comprehensive by respondents. The questionnaire had high internal consistency (alpha = 0.94 at both time points) and test-retest reliability (r = 0.92). Moderately high correlations were found between the RAQoL and physical mobility, pain and energy level sections of the NHP, providing evidence of convergent validity. The RAQoL was able to distinguish between patients grouped by perceived general health, incidence of flare-up and disease severity. The Serbian language version of the RAQoL showed strong evidence of reliability and validity and is recommended for use in clinical trials and routine general practice in RA.

Asunto(s)

Artritis Reumatoide/psicología , Calidad de Vida/psicología , Adulto , Personas con Discapacidad/psicología , Femenino , Humanos , Masculino , Psicometría , Reproducibilidad de los Resultados , Serbia , Encuestas y Cuestionarios , Traducciones

Asunto(s)

Espondiloartritis/epidemiología , Adulto , Anciano , Femenino , Encuestas Epidemiológicas , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Serbia/epidemiología

RESUMEN

The aim of this study was to estimate the prevalence of rheumatoid arthritis (RA) in Serbia, using the European EULAR project methodology. In a detection phase, a previously translated and validated telephone Questionnaire was used by lay interviewers on 6,213 randomly selected telephone numbers representing urban population from four Serbian towns: Belgrade (north), Cacak, Uzice and Krusevac (south). Patients with suspected RA were called again by a rheumatologist. For patients with self-reported diagnosis and positive symptoms, patient's rheumatologist was contacted to confirm diagnosis; a complete rheumatologist examination was scheduled for those with positive symptoms only. Prevalence estimates were standardised for age and sex in relation to Serbian population (census 2002) and further to French population, according to EULAR project methodology. The response rate was 63.6 % (3,950 respondents). The rheumatologist called 571 people, among whom 23 RA cases were confirmed (21 diagnosed previously and 2 newly diagnosed during the examination). The prevalence was 0.16 % (95 % confidence interval CI 0.01-0.32) for men and 0.51 % (95 % CI 0.26-0.76) for women; a female-to-male ratio 3.18. The overall Serbian standardised prevalence was 0.35 % (95 % CI 0.18-0.52); when standardised on French population 0.34 % (95 % CI 0.17-0.51). The highest age-specific rate was in the 65-74-year age band. The EULAR prevalence study, conducted with similar methodology and design, showed that RA prevalence estimates in Serbia (0.34 %) were in accordance with France (0.31 %), but lower than in Lithuania (0.55 %).

Asunto(s)

Artritis Reumatoide/epidemiología , Adolescente , Adulto , Distribución por Edad , Factores de Edad , Anciano , Anciano de 80 o más Años , Artritis Reumatoide/diagnóstico , Femenino , Encuestas Epidemiológicas , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Serbia/epidemiología , Distribución por Sexo , Factores Sexuales , Encuestas y Cuestionarios , Adulto Joven

RESUMEN

OBJECTIVES: to evaluate and compare Moll and Wright, ESSG and CASPAR criteria for psoriatic arthritis (PsA) classification. PATIENTS AND METHODS: Study comprised 356 patients (pts): 120 PsA pts in the investigated group, 123 pts with rheumatoid arthritis (RA) and 113 pts with non-inflammatory musculoskeletal symptoms (NIMS) in two control groups. Clinical diagnosis was the gold standard. Moll and Wright, ESSG and CASPAR criteria were applied to all pts. Sensitivity was calculated for each of the classification criteria sets; specificity was assessed in relation to RA and to NIMS groups, separately. Concordance between the investigated criteria sets was determined. RESULTS: Sensitivity was 91.7% for CASPAR, 85.8% for Moll and Wright and 63.3% for ESSG criteria. Specificity for Moll and Wright criteria was 100%, with relation to both RA and to NIMS group. Specificity of CASPAR criteria was 99.2% and 99.1%; specificity of ESSG criteria was 94.3% and 67%, with regard to RA and to NIMS groups, respectively. Significant fair concordance was found only between CASPAR and Moll and Wright criteria (k=0.379 p<0.001). CONCLUSIONS: The highest sensitivity had the CASPAR criteria, followed by Moll and Wright and ESSG. The highest specificity showed Moll and Wright criteria, followed by CASPAR and ESSG. CASPAR criteria demonstrated high specificity when applied to both NIMS and RA group. The lowest specificity was found for the ESSG criteria in relation to NIMS group. The only significant concordance was shown between CASPAR and Moll and Wright criteria.

Asunto(s)

Artritis Psoriásica/clasificación , Artritis Psoriásica/diagnóstico , Femenino , Humanos , Masculino , Persona de Mediana Edad

RESUMEN

INTRODUCTION: Sjögren's syndrome (SS) is an autoimmune disease of unknown etiology, clinically manifested by dry eyes (xerophthalmia) and dry mouth (xerostomia). In childhood SS is a rare disease, clinically atypically or asymptomatic and is often unrecognized. We report a girl with asymptomatic, juvenile form of primary Sjögren's syndrome (JSS). CASE OUTLINE: A 13-year-old girl was initially observed for several months due to elevated sedimentation rate (ESR 75-90 mm/h) without signs of inflammation or other symptoms and disease signs. Subjective symptoms of dryness of the eyes and mouth were absent at the beginning. Ophthalmologic examination demonstrated hypolacrimia although the patients had no subjective signs of xerophthalmia. Ultrasonography (US) revealed mild enlargement and heterogeneity of large salivary glands parenchyma. Increased rheumatoid factor (RF), anti SS-A/Ro, anti SS-B/La antibodies were found in serum. Ophthalmologic examination demonstrated decreased lacrimation.JSS was confirmed on the basis of ophthalmologic examination, immunological tests, histological findings of biopsy of small and US of major salivary glands. During a 12-years follow-up period systemic or extraglandular manifestations of JSS and other autoimmune diseases were not observed. CONCLUSION: Our experience suggests that in the differential diagnosis of unexplained elevated ESR the primary form of JSS should be also taken into consideration. Ultrasonographic changes of major salivary glands in the absence of symptoms of xerostomia point out that this noninvasive method has an important role in the diagnosis and management of patients with JSS.

Asunto(s)

Síndrome de Sjögren/diagnóstico , Adolescente , Femenino , Humanos

RESUMEN

To assess the prognostic value of the age at onset of Raynaud's (RP) and of a history of exacerbation of RP attacks for the development of connective tissue disease (CTD) in patients initially found to have primary Raynaud's. 3,035 patients with primary RP (2,702 women and 333 men) were followed for an average of 4.8 years (range from 1 to 10 years). At baseline and every 6 months, they were screened for signs and symptoms of CTD. At 4.8 years of follow-up, 54.7 % patients remained as primary RP, 8.1 % had developed suspected secondary RP, and 37.2 % had developed a definite CTD. Primary RP patients had an earlier onset of RP (mean age of 32.2 years) than those with suspected (mean age 36.5 years, P = .007) or definite secondary RP associated with CTD (mean age of 39.8 years, P = .004). RP beginning before the age of forty was not significantly associated with the development of CTD. Conversely, the appearance of RP after the age of 40 was significantly associated with the development of CTD (P = .00001). Worsening of RP attacks predicted the development of CTD, especially systemic sclerosis (relative risk [RR] of 1.42), scleroderma overlap syndrome (RR of 1.18), and mixed CTD (RR of 1.18). Patients whose onset of RP occurred past 40 years of age and those with worsening RP attacks were at risk for the future development of CTD.

Asunto(s)

Enfermedades del Tejido Conjuntivo/etiología , Enfermedad de Raynaud/diagnóstico , Adulto , Edad de Inicio , Enfermedades del Tejido Conjuntivo/diagnóstico , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Enfermedad de Raynaud/complicaciones , Estudios Retrospectivos , Riesgo , Índice de Severidad de la Enfermedad

RESUMEN

INTRODUCTION: Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease associated with decreased functional capacity and potentially long-term consequences. The establishment of early prognostic factors could help in the prevention of joint damage and improve the quality of life in children with JIA. OBJECTIVE: The aim of the study was to evaluate the functional status of children with JIA by using the Childhood Health Assessment Questionnaire (CHAQ) and to assess its ability in predicting the outcome of the disease. METHODS: The study included 87 patients, average age 14 years, under follow-up on the average of 3.7 years. Parents/ patients over 12 years completed CHAQ based on which disability index (DI) was calculated. Disease outcome was determined according to the preliminary criteria for clinical remission. RESULTS: At the end of the follow-up period, functional ability improved significantly (0.541 vs. 0.398; p<0.05). During the study, in 52.8% of patients treatment with biologic drug etanercept was introduced. CHAQ showed good predictive validity when a baseline DI was compared with disease outcome at the final examination (F=18.349; p<0.001). Using the patients with normal functional ability (DI=0) as the reference group, in patients with moderate and more severe functional disability the odds ratio for the disease to be active at the end of the follow-up period (DI> or =0.6) was 4.6 (p=0.044). CONCLUSION: Functional ability of patients with JIA significantly improved during the follow-up period owing to the efficient therapy. The questionnaire for the assessment of the functional status is a simple tool, with good ability to predict disease outcome, and should thus be used in everyday clinical practice with the aim to identify patients with poor prognosis.

Asunto(s)

Artritis Juvenil/diagnóstico , Evaluación de la Discapacidad , Encuestas y Cuestionarios , Adolescente , Artritis Juvenil/terapia , Niño , Preescolar , Femenino , Estado de Salud , Humanos , Masculino , Pronóstico , Adulto Joven

RESUMEN

Clinical features of 40 lymphoproliferative neoplasm patients in the setting of systemic autoimmune diseases managed in the Clinic of Hematology during 1994-2006 were analyzed retrospectively. The classification of systemic autoimmune disease patients was as follows: 15 systemic lupus erythematosus--SLE, 11 rheumatoid arthritis--RA, 12 Sjögren's syndrome--SS, 1 scleroderma, and 1 dermatomyositis. Patients comprised 31 women and 9 men of mean age 55 years (range 33-76). Systemic autoimmune diseases preceeded the development of lymphoproliferative neoplasms in 37/40 (92.5%) patients. Mean latency period between the onset of systemic autoimmune diseases and lymphoproliferative neoplasms occurrence was significantly longer in RA (113 months) than in SLE (75 months) and SS patients (65 months)--P < 0.05. The most frequent lymphoproliferative neoplasms were non-Hodgkin's lymphoma--NHL (35/40; 88%), diffuse large B-cell lymphoma (DBCL)--12 (34%), follicular lymphoma (FC)--7 (20%), small lymphocytic (SL), and marginal zone lymphoma (MZL)--5 (14%) each. The primary site of NHL was extranodal in 18/35 (51.5%) cases. Advanced disease on diagnosis (III + IV clinical stages), constitutional symptoms, and bulky disease were diagnosed in 27/35 (77%), 26/35 (74%), and 3/35 (8.5%) patients, respectively. The overall survival (OS) was as follows (months): DBCL-12, FC-63, SLL-60, and MZL-48. There was no association between the lymphoproliferative neoplasm histological subtype and the systemic autoimmune diseases type or antirheumatic treatment P > 0.05. Our findings are in line with earlier reports showing a high proportion of patients with advanced disease, constitutional symptoms, extranodal manifestations, high grade histology, and low OS in the systemic autoimmune diseases setting.

Asunto(s)

Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/patología , Trastornos Linfoproliferativos/complicaciones , Trastornos Linfoproliferativos/patología , Adulto , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Enfermedades Autoinmunes/mortalidad , Femenino , Humanos , Estimación de Kaplan-Meier , Trastornos Linfoproliferativos/mortalidad , Masculino , Persona de Mediana Edad , Estudios Retrospectivos

RESUMEN

To assess the prognostic value of scleroderma pattern of nailfold capillary changes for the development of connective tissue diseases (CTD) in subjects with primary Raynaud's phenomenon (RP). The study included 3,029 consecutive patients with primary RP who had been followed at 6-month intervals during the mean of 4.8 years. The pathological features of nailfold capillaroscopy were recorded in all patients who had neither clinical nor serological signs of a CTD. In patients who developed CTD, capillary changes obtained 6 months prior to diagnosis were analyzed. A possible relationship between capillary changes and the presence of associated CTD was assessed. At the end of follow-up, 1,660 (54,8%) patients have still the primary RP, 246 (8,1%) had suspected secondary RP, and 1,123 (37,1%) patients developed CTD (363 undifferentiated CTD, 263 systemic sclerosis, 143 systemic lupus erythematosus, 106 rheumatoid arthritis, 102 Sjögren's syndrome, 61 overlap syndrome, 30 vasculitides, 24 mixed CTD, 19 polymyositis, 7 dermatomyositis, and 5 primary antiphospholipid syndrome). Scleroderma pattern were significantly associated with the development of systemic sclerosis [P = .00001, sensitivity 94%, specificity 92%, positive predictive value 52%, negative predictive value 99%, and odds ratio 163 (95% CI, 97,9-271,5)], as well as dermatomyositis (P = .0004), overlap syndrome with signs of systemic sclerosis (P = .0001), and mixed connective tissue disease (P = .007). Capillary microscopy is effective method for differentiation between primary and secondary RP and useful tool for the prediction of scleroderma spectrum disorders in RP patients.

Asunto(s)

Capilares/patología , Enfermedades del Tejido Conjuntivo/etiología , Uñas/irrigación sanguínea , Enfermedad de Raynaud/complicaciones , Esclerodermia Sistémica/etiología , Adolescente , Adulto , Anciano , Distribución de Chi-Cuadrado , Niño , Enfermedades del Tejido Conjuntivo/patología , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Angioscopía Microscópica , Persona de Mediana Edad , Oportunidad Relativa , Valor Predictivo de las Pruebas , Pronóstico , Estudios Prospectivos , Enfermedad de Raynaud/patología , Medición de Riesgo , Factores de Riesgo , Esclerodermia Sistémica/patología , Sensibilidad y Especificidad , Factores de Tiempo , Adulto Joven

Asunto(s)

Artritis Psoriásica/clasificación , Artritis Psoriásica/diagnóstico , Guías de Práctica Clínica como Asunto/normas , Reumatología/normas , Humanos , Sensibilidad y Especificidad

RESUMEN

INTRODUCTION: It is well known that juvenile idiopathic arthritis (JIA) as a chronic inflammatory disease with onset during the childhood, beside other complication, can lead to bone metabolism disturbance and osteoporosis. OBJECTIVE: To assess bone mineral density (BMD) in children with JIA and to identify factors playing role in bone mineral disturbance. METHODS: Seventy-five patients (26 male and 49 female) average disease duration 7.2 (2.4-16.8) years, and 73 age matched healthy control subjects (29 male and 44 female) participated in the study. Mean age of the groups was about 14.5 years. BMD was determined by dual x-ray absorptiometry (DEXA) of the lumbar spine (L2-L4). For further analysis we used the absolute value of BMD, expressed as g/cm2, Z score expressed as SD (relative value as standard deviation decline of normal BMD values of referent Italian population with identical age and gender), bone mineral content (BMC) as g/cm, and corrected BMD-BMDv as g/cm3. RESULTS: Z score in the group of patients was significantly lower (-1.02 +/- 1.6) in comparison to the control group (-0.09 +/- 1.4; p<0.001). BMD, BMDv and BMC were also statistically lower in patients with JIA. The lowest Z score was found in patients with systemic onset (-2.63 SD). Z score showed a statistically significant positive correlation with arthritis course (polyarticular course had lower Z score), body mass index and standard deviation score for height and weight. Statistically significant negative correlation was detected in regard to Z score and glucocorticoid (GC) treatment duration, GC cumulative dose, number of joints with limited range of motion, radiological stage and functional class. CONCLUSION: The results showed a decreased BMD in patients with JIA in comparison to the control group. Systemic onset, polyarthritis, longer treatment with GC and higher cumulative dosage, as well as higher damage level (functional status and radiological stage) are factors playing negative role in bone metabolism in children with JIA.

Asunto(s)

Artritis Juvenil/patología , Densidad Ósea , Absorciometría de Fotón , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino

RESUMEN

To assess the prognostic value of capillaroscopy findings for the development of connective tissue disease in children and adolescents with Raynaud phenomenon, we followed up a group of 250 (mean age 15 years) for 1 to 6 years after the first capillaroscopy was performed. Every 6 months they were screened for signs and symptoms of connective tissue disease. Analysis was performed on capillary changes registered 6 months before the development of connective tissue disease. Capillary changes were classified into three types: normal, nonspecific, and sclerodermatous. At the end of the follow-up period, 191 (76%) subjects had primary Raynaud phenomenon, 27 (10.8%) were diagnosed as having undifferentiated connective tissue disease, and 32 (12.8%) fulfilled the criteria for a diagnosis of a specific connective tissue disease. Systemic lupus erythematosus was found in nine (3.6%) patients, rheumatoid arthritis in 10 (4%) patients (six of them with juvenile onset rheumatoid arthritis), and scleroderma spectrum disorders in 13 (5.2%). The mean time for the evolution of Raynaud phenomenon into undifferentiated connective tissue disease or a form of the disease was 2 years. Most of the subjects with primary Raynaud phenomenon (173/191, 91%), undifferentiated connective tissue disease (22/27, 81%), juvenile onset rheumatoid arthritis/rheumatoid arthritis (7/10, 70%), and systemic lupus erythematosus (6/9, 67%) had normal capillary findings. Nonspecific capillary changes occurred in 3 of 10 (30%) patients with rheumatoid arthritis, 2 of 9 (22%) with systemic lupus erythematosus, 4 of 27 (15%) with undifferentiated connective tissue disease, and 18 of 191 (9%) with primary Raynaud phenomenon. Of all the subjects, only 10 (4%) showed sclerodermatous disease type capillary changes 6 months before the expression of a particular disease: eight (62%) of these developed scleroderma spectrum disorders, one expressed systemic lupus erythematosus, and one had undifferentiated connective tissue disease. We concluded that there were no specific capillary changes predictive for future development of systemic lupus erythematosus, juvenile onset rheumatoid arthritis/rheumatoid arthritis, and undifferentiated connective tissue disease in children and adolescents with Raynaud phenomenon. Most of our study subjects with Raynaud phenomenon who developed these diseases had normal capillary findings or nonspecific changes. Children and adolescents who developed scleroderma spectrum disorders showed a sclerodermatous type of capillary changes 6 months before the expression of the disease, indicating that this type of capillary changes in children and adolescents with Raynaud phenomenon highly correlated with further development of scleroderma spectrum disorders.

Asunto(s)

Enfermedades del Tejido Conjuntivo/etiología , Uñas/irrigación sanguínea , Enfermedad de Raynaud/diagnóstico , Enfermedad de Raynaud/fisiopatología , Adolescente , Capilares/fisiopatología , Niño , Enfermedades del Tejido Conjuntivo/diagnóstico , Enfermedades del Tejido Conjuntivo/fisiopatología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Angioscopía Microscópica , Valor Predictivo de las Pruebas , Pronóstico , Enfermedad de Raynaud/complicaciones , Factores de Tiempo

RESUMEN

In this paper we report data regarding the IgM Y7 cross-reactive idiotope (CRIo) obtained by analysis of: 1) its V-gene subgroup dependance, 2) the frequency of its expression on human monoclonal IgMs and IgM molecules from normal and pathological sera. Furthermore, comparison of epitopic repertoire and nature of binding of human monoclonal IgMs expressing Y7 CRIo was performed to confirm the natural antibody properties of these molecules. IgM isolated from sera of patient DJ (IgM DJ) which expresses the Y7 idiotope has been classified to VH3/VL2 subgroup. From ten IgMs tested only IgM from patient RD (IgM RD) has been shown to express Y7 idiotope. Y7+ human IgMs bound to ssDNA, lactic acid bacteria, mouse laminin, porcine thyroglobulin and mouse IgG. Higher percentage of the expression of Y7 CRIo was detected in the sera of patients suffering from autoimmune diseases such as lupus, rheumatoid arthritis and psoriasis vulgaris as well as in patients suffering from chronic infections of the lower urinary tract. Antigen binding repertoire and properties of Y7+ monoclonal IgM, frequency of Y7 expression on monoclonal IgMs and its concentration in normal and pathological sera indicate the important biological role of this CRIo within the immune system.

Asunto(s)

Idiotipos de Inmunoglobulinas/análisis , Inmunoglobulina M/inmunología , Macroglobulinemia de Waldenström/inmunología , Secuencia de Aminoácidos , Anticuerpos Monoclonales/genética , Anticuerpos Monoclonales/inmunología , Afinidad de Anticuerpos , Enfermedad Crónica , Reacciones Cruzadas , Ensayo de Inmunoadsorción Enzimática , Epítopos/análisis , Humanos , Inmunoglobulina M/genética , Datos de Secuencia Molecular , Macroglobulinemia de Waldenström/sangre

RESUMEN

The study carried out was at the Department of Nephrology, Military Medical Academy, over the period from 1996 to 2001. Different types of lupus nephritis were documented in 42 patients and were treated with standard therapeutic protocols (corticosteroids, the pulse dose of cyclophosphamide + corticosteroids) and cyclosporine in the target serum concentration of 100-120 ng/ml along with pronisone of 15-20 mg per day. The different degree of damaged renal function was observed. Renal biopsy was performed in 13 patients and in one patient rebiopsy was done. Twenty one patients were treated only with corticosteroids (remission in 23.8% of cases), with cyclophosphamide + corticosteroids 33 patients (remission in 42.4% of cases) and cyclosporine + corticosteroids 12 patients (remission in 91.7% of cases). The pulse therapy with cyclophosphamide in combination with corticosteroids, and cyclosporine in combination with lower doses of corticosteroids was statistically more successful in comparison with corticosteroids monotherapy (p < 0.01). Remission was found in 73.8% of patients, terminal renal weakness was observed in 7 patients, and fatal outcome in 4 patients. We recommend the pulse therapy of cyclophosphamide in combination with corticosteroids in the treatment of severe clinical forms of lupus nephritis, and in refractive forms cyclosporine in combination with low doses of corticosteroids.

Asunto(s)

Ciclosporina/administración & dosificación , Glucocorticoides/administración & dosificación , Inmunosupresores/administración & dosificación , Nefritis Lúpica/tratamiento farmacológico , Adulto , Quimioterapia Combinada , Femenino , Humanos , Masculino , Quimioterapia por Pulso

RESUMEN

INTRODUCTION: Immunosuppressive drugs, particularly cyclophosphamide, are widely accepted as the treatment of choice for severe, proliferative lupus nephritis. However, there is no consensus with regard to: 1) the dose required for achieving control of disease activity; 2) duration of cyclophosphamide therapy after the achievement of treatment response; 3) treatment of lupus nephritis relapses [1-5]. In the Institute of Rheumatology, Belgrade, two regimens of intravenous cyclophosphamide have been introduced in the treatment of lupus nephritis patients years ago. The first has comprised the so called "small pulses" that have been used since 1985, and the second has been standard protocol with high doses of cyclophosphamide, accepted in 1990. Results of these follow-up studies were published previously [6-8]. AIM: The aim of this study was to compare the efficacy of two regimens of intravenous pulse cyclophosphamide in the treatment of patients with severe lupus nephritis. METHODS: We analyzed the results of two follow-up studies comprising patients with lupus nephritis, treated with cyclophosphamide: 1) 41 females treated with "small pulses", consisting of 400 mg of cyclophosphamide weekly at treatment onset, followed by the same dose fortnightly for the next three months, and finally on monthly basis for several months or years; 2) 33 patients (29 females and 4 males) treated with standard protocol consisting of "induction phase" with 6 monthly pulses of high doses (0.5-0.75 g/m2 body surface), followed by "maintenance phase" with quarterly pulses for additional 1-2 years. The evaluation of long-term treatment effects was based on remission/response rate [9], number of patients with renal failure, end-stage renal disease and death outcome. RESULTS: Groups of patients were quite comparable with respect to their demographic and clinical data (Table 1). The only difference was much higher frequency of renal biopsy in "high dose" cyclophosphamide pulse (85% versus 32%), confirming the presence of proliferative lupus nephritis. Cummulative dose of cyclophosphamide and treatment duration were not significantly different between treatment groups. At the end of the follow-up, distributions of favorable (remission/response) and unfavorable outcome was similar (p = 0.831; Mann-Whitney U test), as well as dynamics of remission achieving (p = 0.068; Log-rank test), cummulative renal survival (p = 0.129; Log-rank test) and patient survival (p = 0.577; Log-rank test). DISCUSSION: Similar efficacy of two different cyclophosphamide regimens in our patients with lupus nephritis was not surprising considering that cummulative cyclophosphamide doses and treatment duration were similar obtaining similar control of disease. During induction phase of treatment, patients on small pulses have received even higher cummulative dose of cyclophosphamide. Aggressive immunosuppressive treatment with cyclophosphamide has significantly ameliorated the outcome of lupus nephritis. In different studies, rate of assessed clinical response is 60-80 [13-17]. Significant proportion (42%) of patients who achieved partial remission, as well as complete remission, developed flare of renal disease several months after the end of the treatment, necessitating restarting of pulse cyclophosphamide therapy. The results of our study were in accordance with those results, especially with results of Mosca et al. [18] who have applied the duration of treatment similar to ours in high pulse regimen. CONCLUSION: Treatment response did not differ between two different cyclophosphamide regimens (small pulses and standard high doses protocol), but standard protocol seemed to be more comfortable for patients. We recommend standard protocol for patients with biopsy proved proliferative lupus nephritis as a gold treatment standard. However, sustained remission of proliferative lupus nephritis is a goal that still remains to be achieved.

Asunto(s)

Ciclofosfamida/administración & dosificación , Inmunosupresores/administración & dosificación , Nefritis Lúpica/tratamiento farmacológico , Adolescente , Adulto , Niño , Esquema de Medicación , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Quimioterapia por Pulso