In our study, we collected data on 116 patients with biopsy-proven idiopathic or lupus glomerulonephritis who were treated with high doses of intravenous immunoglobulin G (IVIG) (Veinoglobuline or Immunovenin-intact). In all patients a severe nephrotic syndrome (edema, proteinuria >6 g/24 h, serum albumin <22 g/24 h) had been observed. 34 patients had renal failure (serum creatinine up to 504 µmol/l) and 96 hypertension. 98 patients were previously for a long time treated with corticosteroids, immunosuppressors and anticoagulants without any effect. 18 patients had no therapy before IVIG. IVIG had been applied in a dose of 85 mg/kg/24 h 3 times every other day. Depending on the clinical improvement afterwards (in case of therapy resistance or relapse) these boli had been repeated in 84 patients after 1 month (and every 3 months for maintenance of remission) to 7 years. Proteinuria disappeared and full remission occurred in 36 patients. Partial remission was present in 48 patients. 32 patients went into end-stage renal failure and/or died (15 of them of a nonrenal cause). In 13/34 patients with impaired renal function serum creatinine levels go back to normal after treatment. Our results suggested that IVIG therapy may be recommended in patients unresponsive to aggressive conventional treatment.

The promoter polymorphism −174G/C within the interleukin-6 gene (IL-6) has been reported to have a functional importance through the modulation of IL-6 gene expression in vitro and in vivo. IL-6 is thought to play an important role in autoimmune diseases and the effect of its receptor inhibitor—tocilizumab—has been recently studied. The aim of this case-control study was to investigate the association between the interleukin-6 −174G/C single nucleotide polymorphism and the susceptibility to dermatomyositis (DM) and systemic lupus erythematosus (SLE) in Bulgarian patients. Altogether, 87 patients—52 with SLE and 35 with DM—as well as 80 unrelated healthy controls were included in this study. All of them were analyzed by restriction fragment length polymorphism analysis (RFLP). The GG genotype and the G allele appeared to be associated with SLE, especially in women. None of the genotypes showed an association with DM. However, the G allele appeared to be associated with muscle weakness and it is a risk factor for elevated muscle enzymes. Our results indicate that IL-6 −174G/C polymorphism might be associated with the susceptibility to SLE especially in women. Although it is not associated with DM, it seems that IL-6 −174G/C polymorphism could modulate some clinical features in the autoimmune myopathies.

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Rationale:Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by autoantibody production, complement activation, and deposition of immune complexes in tissues and organs. SLE can involve any region of the visual system. Although ocular manifestations are not part of the classification criteria for SLE, they can be observed in up to one-third of the patients with SLE. They are rarely reported at the time of disease onset. Retinal vasculitis is usually associated with active generalized disease. Due to its low frequency, we report a case of acute necrotizing retinal vasculitis as onset of SLE.Patient concerns and diagnosis:A 25-year-old white female was referred to the rheumatology clinic with gradually and rapid deterioration of the vision due to abnormal vessel permeability in the right fundus with edema along the vessels, occlusion of arterial branches in the middle periphery with leakage of the dye in these areas and indentical but less prominent changes with cotton wool spots in the papillomacular area and extensive hemorrhages in the left eye. The onset of malar rash, arthralgias and positive antinuclear, anti-double stranded DNA, anti-ribosomal P and anti-β2 glycoprotein I antibodies with decreased C4 complement levels, as well as the positive lupus-band test confirmed the diagnosis of SLE.Interventions:Aggressive immunomodulating therapy with high-dose methylprednisolone, intravenous immunoglobulin, and cyclophosphamide was used for suppression of the disease activity followed by azathioprine as maintaince therapy.Outcomes:Substantial improvement and partial resorption of the vasculitic changes, including central retinal artery and vein, was achieved prominently in the left eye. The study was conducted in accordance with the Declaration of Helsinki and written informed consent was obtained from the patient. Because of this, there is no need to conduct special ethic review and the ethical approval is not necessary.Lessons:Inclusion of ocular manifestations among the classification criteria for SLE would enable earlier establishment of the diagnosis and therapeutic interventions in some instances of SLE.

BackgroundSystemic sclerosis (SSc) is a chronic, autoimmune disease of the connective tissue that involves skin, subcutaneous tissue, muscles and joints, as well as the internal organs: kidneys, lungs, heart. Depending on the extent it can occur as limited (lcSSc) or diffuse (dcSSc) clinical variant. About half of the patients with renal involvement the clinical manifestation is limited to a moderate increase in serum creatinine, mild proteinuria, and moderate hypertension. The most serious complication remains sclerodermal renal crisis (SRC). There was significant heterogeneity in definitions. As a rule for SRC was characterised by new-onset severe hypertension, hypertensive encephalopathy and seizures, acute kidney injury with oliguria, proteinuria and erythrocyturia, and microangiopathic hemolytic anaemia with thrombocytopenia.ObjectivesThe aim of our study was to analyse the clinical features and outcome of patients with scleroderma renal crisis.MethodsWe retrospectively reviewed the medical records of patients with scleroderma renal crisis between January 2002 and December 2016. Conditional logistic regression and multivariate analysis was performed to determine factors independently associated with outcome in patients with SRC.Results18 (14 females and 4 males, median age 47,3±14,6 years) patients diagnosed with SRC were included in the study. The mean duration from first symptom of systemic sclerosis (Raynaud’s phenomenon, cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease, pulmonary arterial hypertension, digestive hypomotility and etc.) to SRC attack was 4,47±2,9 years. 13 SRC patients belonged to dcSSc, and 5 patients – to lcSSc. Among SRC patients, 9 were negative of anti-centromere antibodies (ACAs). All these 18 patients had hypertension and renal insufficiency, including 4 dialysis dependent after the onset of SRC and 6 with thrombotic microangiopathy. There were 6 patients receiving renal biopsy (the pathological findings were mainly summarised as intimal thickening and stenosis of renal arterioles). Among 16 patients with long-term followed-up, 15 patients received angiotensin converting enzyme inhibitors (ACEI), 8 patients died, 1 patient was dialysis dependent. Survival from first symptom in those patients with Raynaud’s phenomenon mode of onset was higher at 5 years and at 10 years than those with onset as non-Raynaud’s phenomenon (p < 0,05). In multivariate analysis, factors related to mortality in SRC were older age at onset, male gender, treatment with corticosteroids, dcSSc subset, interstitial lung disease, pulmonary arterial hypertension, heart involvement, and the mode of onset with non-Raynaud’s phenomenon, especially in the form of pulmonary involvement. The mode of onset should be considered an independent prognostic factor in SRS. The treatment of SRC relies on aggressive blood pressure control with an ACEI, combined with other antihypertensive drugs if needed.ConclusionsSRC usually occurred at the early course of SSc. dcSSc was more frequent than lcSSc....