Low-Density Lipoprotein Apheresis in Patients with Acute Kidney Injury Due to Minimal Change Disease Requiring Acute Renal Replacement Therapy.

Low-density lipoprotein apheresis (LDL-A) has been developed as a therapy for familial hypercholesterolemia, but LDL-A has also been used as a general treatment for drug-resistant nephrotic syndrome (NS) due to focal segmental glomerulosclerosis (FSGS). The patients with NS due to minimal change disease (MCD) are often difficult to control effective circulating plasma volume, causes acute kidney injury (AKI), and when diuretics are not effective and the respiratory condition of patients worsens, patients require acute renal replacement therapy (ARRT). The effectiveness of LDL-A is not only reduction of serum low-density lipoprotein but also various other benefits. LDL-A might have improved renal hemodynamics by reducing vasoconstrictive eicosanoids and contributed to the therapeutic effect of antiproteinuric drugs such as corticosteroids. We treated a 49-year-old Japanese woman and a 71-year-old Japanese man with AKI caused by NS due to MCD, who required ARRT. Although these patients received ARRT and corticosteroids, their AKI and MCD did not improve sufficiently. We initiated LDL-A treatment for these patients as an additional treatment modality, because their total serum cholesterol levels were high at the time of admission. After the additional LDL-A treatment, both patients were able to discontinue ARRT, because NS and AKI in both patients were improved sufficiently. It is possible that early additional LDL-A is effective for patients with AKI and NS due to MCD who require ARRT, and may help patients discontinue ARRT because of the effect of LDL-A such as improving hypercoagulability and renal hemodynamics and contributing to the therapeutic effect of corticosteroids.

A case of membranous nephropathy diagnosed with lupus nephritis 11 years after onset.

A 34-year-old female patient presented to our hospital with lower extremity edema and proteinuria during pregnancy. Renal biopsy was performed and the patient was diagnosed with nephrotic syndrome due to lupus-like membranous nephropathy. This diagnosis was reached upon as laboratory findings upon admission, wherein both anti-nuclear and anti-double-stranded DNA antibodies revealed negative, did not fulfill the criteria for systemic lupus erythematosus (SLE) proposed by the American College of Rheumatology (ACR) and the patient did not reveal any typical physical manifestations of SLE. Methylprednisolone pulse therapy was started followed by oral administration of prednisolone. Urinary protein excretion diminished after 1 year of treatment. Eleven years later, the same patient was admitted to our hospital again with relapse of nephrotic syndrome. Laboratory findings upon second admission, wherein both anti-nuclear and anti-double-stranded DNA antibodies revealed positive, fulfilled the ACR criteria. Renal biopsy was performed again, resulting in a diagnosis of lupus nephritis. Steroid therapy combined with administration of mycophenolate mofetil led to an incomplete remission. Immunofluorescence studies confirmed the presence of IgG, IgM, C3, and C1q in renal biopsy specimens both at first and second admissions. Furthermore, immunofluorescence studies confirmed the presence of IgG1-4 in the first biopsy and tubuloreticular inclusions (TRIs) were revealed using electron microscopy. The present case represents the possibility that characteristic pathological findings of lupus nephritis, including TRIs, can reveal themselves before a diagnosis of SLE.

Efficacy of a Moisturizer for Pruritus Accompanied With Asteatosis in Dialysis Patients: An Open-Label, Randomized, Exploratory Study.

BACKGROUND: In dialysis patients, skin disorders (dryness and itching) are frequently observed and treated with a moisturizer, in the absence of clear evidence of efficacy. STUDY DESIGN: An open-label, randomized, before/after, parallel-group, comparative/exploratory study. SETTING & PARTICIPANTS: 12 Japanese patients with chronic kidney failure undergoing maintenance hemodialysis who presented with dry skin and itching. INTERVENTION: Patients received a topical heparinoid moisturizer as the study drug for 2 weeks from the first day of the study treatment, followed by either a 2-week washout (group A: 6 participants) or further 2-week treatment (group B: 6 participants). OUTCOMES: The primary end point was change in water content in the stratum corneum in the hypochondrium. Secondary end points included change in visual analogue scale itching score and subjective evaluations of symptoms. To evaluate safety, adverse events were also investigated. MEASUREMENTS: Water content of the stratum corneum, dryness/itching improvement rating, itching visual analogue scale/duration of itching, photographic evaluation of skin symptoms, principal investigator's overall assessment of study drug, and adverse events. RESULTS: Mean water content of the stratum corneum in the combined groups significantly increased at week 2 (51.2 arbitrary units [AU] vs treatment start day, 31.6 AU; P<0.001), but significantly decreased at week 4 in group A, in which patients discontinued treatment with the study drug (39.4 AU; P = 0.005). Other efficacy end points, including the visual analogue scale itching score, were also improved by treatment with the study drug, but such improvement was not sustained after discontinuation of treatment. There were no adverse events related to the study treatment. LIMITATIONS: Only Japanese patients were included in the study, with a small sample size. CONCLUSIONS: Continuous application of the topical heparinoid moisturizer increased water content in the stratum corneum and lessened itching in dialysis patients. FUNDING: Maruho Co, Ltd. TRIAL REGISTRATION: Registered at the University Hospital Medical Information Network Clinical Trials Registry with study number UMIN000017016.

A Case of Anti-Glomerular Basement Membrane Antibody-Positive Systemic Lupus Erythematosus with Pulmonary Hemorrhage Successfully Treated at an Early Stage of the Disease.

We report here a case of systemic lupus erythematosus (SLE) with pulmonary hemorrhage and anti-glomerular basement membrane (anti-GBM) antibodies. A 42-year-old woman was admitted to our hospital with complaints of exanthema, arthralgia, shortness of breath, and hemoptysis. Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage. Laboratory findings on admission revealed proteinuria, microscopic hematuria, anemia, leukopenia, hypoalbuminemia, hypocomplementemia, and slightly elevated levels of serum creatinine. Serological tests revealed elevated titers of serum anti-GBM antibodies, proteinase 3-antineutrophil cytoplasmic antibodies (PR3-ANCA), and anti-double stranded deoxyribonucleic acid (dsDNA)-immunoglobulin G (IgG) antibodies. Early treatment with steroid pulse therapy combined with plasma exchange resolved the patient's pulmonary hemorrhage and renal dysfunction. Renal biopsy carried out after the treatment revealed a recovery phase of acute tubular injury with minor glomerular abnormalities without linear IgG deposition along the GBMs. For a good prognosis, it is necessary to start treatment immediately in patients with anti-GBM antibody-positive SLE associated with pulmonary hemorrhage.