Outcome of patients with lupus nephritis under long-term immunonephrological care / Tartós immunonephrológiai gondozásban részesült lupus nephritises betegeink hosszú távú kezelési eredményei

INTRODUCTION: Lupus nephritis is the most severe complication of systemic lupus erythematosus (SLE), its development and the effectiveness of immunosuppressive therapy substantially influence patients' quality of life and survival. AIM: In this retrospective observational investigation, the long term-outcome of patients with lupus nephritis, followed at the St. Margit Hospital Immunonephrological Outpatient Clinic, was evaluated. RESULTS: Between 1997 December 1 and 2019 April 30, 73 patients (age 33.7 ± 15 years, 82% female, 18% male) were under care with median observation of 119 [between 3-264] months. At diagnosis, eGFR showed 68 [7-120] ml/min, proteinuria was 2800 [23-16812] mg/day; 10 patients needed dialysis treatment acutely. Renal biopsy, performed in 68 patients, proved proliferative lupus nephritis in 55 and pure membranous lupus nephritis in 6 patients. Administering combined immunosuppressive therapy, complete remission was achieved in 50 and partial remission in 21 cases; one or repeated relapses developed in 28 subjects. Two patients, by the time they got under our care, had already required chronic dialysis, and in the long term, three more patients progressed to end-stage renal disease requiring renal replacement therapy. Renal function stabilized in all other participants, clinical activity of SLE, SLEDAI score, complement levels and immunserology results improved significantly. CONCLUSIONS: Lupus nephritis can be effectively treated by combined induction and prolonged maintenance immunosuppression, but to prevent progression of the disease, long-term care is necessary by co-operation of nephrologist and immunologist. To provide adequate prevention and therapy of the SLE's multiorgan involvement and also the potential complications of immunosuppression, multidisciplinary team is needed with all specialists who may facilitate these patients' complex care. For the long-term management of patients with lupus nephritis, the nephrologists have to be responsible, and the multidisciplinary teams also have to be under their direction. Orv Hetil. 2020; 161(31): 1293-1301.

Simple, readily available clinical indices predict early and late mortality among patients with ANCA-associated vasculitis.

BACKGROUND: The early identification of patients with ANCA-associated vasculitis (AAV) who are at increased risk for inferior clinical outcome at the time of diagnosis might help to optimize the immunosuppressive therapy. In this study we wanted to determine the predictive value of simple clinical characteristics, which may be applicable for early risk-stratification of patients with AAV. METHODS: We retrospectively analyzed the outcome of 101 consecutive patients with AAV receiving a protocolized immunosuppressive therapy. Baseline Birmingham Vasculitis Activity Score (BVAS) and non-vasculitic comorbidities were computed, then predictors of early (<90 days) and late (>90 days) mortality, infectious death, relapse and end stage kidney disease (ESKD) were evaluated. RESULTS: The baseline comorbidity score independently predicted early mortality (HR 1.622, CI 1.006-2.614), and showed association with infectious mortality (HR 2.056, CI 1.247-3.392). Patients with BVAS at or above median (=21) had worse early mortality in univariable analysis (HR 3.57, CI 1.039-12.243) (p = 0.031), and had more frequent relapses (p = 0.01) compared to patients with BVAS below median. CONCLUSIONS: Assessing baseline comorbidities, beside clinical indices characterizing the severity and extension of AAV, might help clinicians in risk-stratification of patients. Future prospective studies are needed to investigate whether therapies based on risk-stratification could improve both short term and long term survival.

Clinical outcomes of ANCA-associated vasculitis in elderly patients.

BACKGROUND: Older age is independently associated with mortality in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). We hypothesized that a reduced-dose immunosuppressive treatment would result in similar effectiveness and comparable treatment-related morbidity in elderly patients as the regular dose in younger patients. We also postulated that the higher baseline comorbidities may contribute to the higher mortality of the elderly subjects. METHODS: Ninety-three consecutive patients with AAV between 1998 and 2012 were retrospectively analyzed. Forty-one individuals were defined as "elderly" (age >65 years) and 52 as "younger" (age <65 years). All cause and cardiovascular mortality, death due to vasculitis and infections, and effectiveness of "reduced-dose" immunosuppressive treatment in the elderly group were compared to the effects of "full-dose" treatment in younger individuals. RESULTS: Mortality in the elderly group was higher (p = 0.007). Cardiovascular death was significantly increased (p = 0.002) in the elderly, but mortality due to vasculitis or infections was comparable. Treatment effectiveness was also similar in elderly and younger patients. At the end of the first follow-up year, 37 % of the elderly and 27 % of the younger patients died (p = 0.22). In univariate Cox regression analyses, being older than 65 year, having cardiovascular disease at baseline, need for dialysis at diagnosis, and lower serum albumin were associated with an increased hazard of mortality. CONCLUSIONS: Delivering reduced dose of immunosuppression for elderly patients was associated with satisfactory outcome and favorable treatment-related complication profile. The higher mortality in the elderly could be attributed mainly to baseline cardiovascular morbidity.

[Role of plasmapheresis in immunological kidney diseases. Experience from 1050 completed plasmapheresis treatment sessions]. / A plazmaferézis szerepe immunológiai etiológiájú vesebetegségekben. Tapasztalataink 1050 elvégzett feréziskezelés kapcsán.

UNLABELLED: Plasmapheresis is an effective treatment modality in several immunological kidney diseases. It is also indicated in certain neurological and hematological abnormalities, and some other diseases. AIMS: In this study the indications and outcomes of the plasma exchange treatments performed in the Plasmapheresis Unit of the authors during the last 12 years are summarized, and the findings are compared to those published in the literature. The procedure, mechanisms of action and adverse effects are also briefly discussed. METHODS: Between 1999 and 2010 authors completed 1050 plasma exchanges in 195 patients with an average 5.4 (1-20) treatments/person. In the 78 males and 117 females (age 57±16 years) the indications were as follows: 47% anti-cytoplasmic antibody-associated vasculitis, 4% anti-glomerular basement membrane disease, 3% rapidly progressing immunocomplex glomerulonephritis, 11% severe complications of systemic lupus erythematosus, 1% treatment resistant focal segmental glomerular sclerosis, 5% hemolytic uremic syndrome, 13% complications of multiple myeloma, 4% HELLP syndrome, 10% neurological diseases, and 2% other abnormalities. RESULTS: Plasmapheresis, completed as part of combined immunosuppressive treatment, resulted in remarkable improvements in patients with anti-cytoplasmic antibody-associated vasculitis. Out of the 91 patients, 54 needed urgent dialysis on admission, and renal replacement therapy could be discontinued in 44% of them. Renal functions in those patients who did not need dialysis also improved significantly, and pulmonary hemorrhage ceased in all affected subjects. Survival of the patients with anti-glomerular basement membrane diseases was 100%. The treatment significantly improved the renal function in rapidly progressive lupus nephritis, and all the 5 cases of lupus cerebritis were successfully cured. The results showed less effectiveness in therapy resistant focal segmental glomerular sclerosis and in rapidly progressing immunocomplex glomerulonephritis. Plasmapheresis proved to be very efficient in cases with the primary hemolytic uremic syndrome, and each patient with HELLP syndrome recovered completely. The outcome of those with multiple myeloma was less favorable, although hyperviscosity was rapidly and effectively decreased by the plasmapheresis. The treatment improved the conditions of almost all patients with neurological diseases. DISCUSSION: According to these findings plasmapheresis treatment, introduced by proper indications, effectively improves the outcomes of several diseases. Early diagnosis and immediate introduction of the plasmapheresis are very important - in conjunction with the appropriate therapy of the underlying diseases.