Renal involvement in sarcoidosis: histological patterns and prognosis, an Italian survey.

BACKGROUND: Granulomatous interstitial nephritis in sarcoidosis (sGIN) is generally clinically silent, but in <1% causes acute kidney injury (AKI). METHODS: This Italian multicentric retrospective study included 39 sarcoidosis-patients with renal involvement at renal biopsy: 31 sGIN-AKI, 5 with other patterns (No-sGIN-AKI), 3 with nephrotic proteinuria. We investigate the predictive value of clinical features, laboratory, radiological parameters and histological patterns regarding steroid response. Primary endpoint: incident chronic kidney disease (CKD) beyond the 1°follow-up (FU) year; secondary endpoint: response at 1°line steroid therapy; combined endpoint: the association of initial steroid response and outcome at the end of FU. RESULTS: Complete recovery in all 5 No-sGIN-AKI-patients, only in 45% (13/29) sGIN-AKI-patients (p=0.046) (one lost in follow-up, for another not available renal function after steroids). Nobody had not response. Primary endpoint of 22 sGIN-AKI subjects: 65% (13/20) starting with normal renal function developed CKD (2/22 had basal CKD; median FU 77 months, 15-300). Combined endpoint: 29% (6/21) had complete recovery and final normal renal function (one with renal relapse), 48% (10/21) had partial recovery and final CKD (3 with renal relapse, of whom one with basal CKD) (p=0.024). Acute onset and hypercalcaemia were associated to milder AKI and better recovery than subacute onset and patients without hypercalcaemia, women had better endpoints than men. Giant cells, severe interstitial infiltrate and interstitial fibrosis seemed negative predictors in terms of endpoints. CONCLUSIONS: sGIN-AKI-patients with no complete recovery at 1°line steroid should be treated with other immunosuppressive to avoid CKD, in particular if males with subacute onset and III stage-not hypercalcaemic AKI.

[New scenarios in secondary hyperparathyroidism: etelcalcetide. Position paper of Nephrologists form Lombardy].

Bone mineral abnormalities (defined as Chronic Kidney Disease Mineral Bone Disorder; CKD-MBD) are prevalent and associated with a substantial risk burden and poor prognosis in CKD population. Several lines of evidence support the notion that a large proportion of patients receiving maintenance dialysis experience a suboptimal biochemical control of CKD-MBD. Although no study has ever demonstrated conclusively that CKD-MBD control is associated with improved survival, an expanding therapeutic armamentarium is available to correct bone mineral abnormalities. In this position paper of Lombardy Nephrologists, a summary of the state of art of CKD-MBD as well as a summary of the unmet clinical needs will be provided. Furthermore, this position paper will focus on the potential and drawbacks of a new injectable calcimimetic, etelcalcetide, a drug available in Italy since few months ago.

Lenvatinib-induced renal failure: two first-time case reports and review of literature.

INTRODUCTION: Lenvatinib (LEN) is a multi-kinase anti-angiogenic drug recently approved in several cancers. LEN is not easily manageable due to its complex safety profile. Proteinuria and renal failure (RF) were reported among the most frequent LEN-induced adverse events (AEs), often leading to discontinuations or dose modifications. Understanding the pathogenesis of these AEs could ameliorate the management of LEN-induced renal toxicity. Areas covered: We present two cases of LEN-induced renal failure (LIRF) with different pathogenesis. 1) LIRF with severe proteinuria in a man treated for a metastatic papillary thyroid carcinoma. Kidney biopsy showed a glomerular damage secondary to LEN, having excluded other causes of RF. 2) LIRF without proteinuria in a woman with metastatic adenoid cystic carcinoma of minor salivary gland. A tubulointerstitial nephropathy was supposed by clinical evaluation and laboratory tests. Effective management was obtained by oral steroids without interrupting LEN. Expert opinion: The case 1 presented for the first time the histological picture of LIRF with a classical glomerular damage leading to secondary proteinuria and tubular failure. Case 2 showed an alternative LIRF pattern of likely tubulointerstitial injury without proteinuria. These reports reflect two sides of the same coin, both to be considered in case of LIRF.

Change gout: how to deal with this "silently-developing killer" in everyday clinical practice.

BACKGROUND: Despite being regarded as an easily-treatable disease, gout diagnosis and management can be challenging. REVIEW: This review discusses current issues in gout management and proposes some potential solutions. Gout diagnosis should be reached as early as possible and often requires specific tests, such as synovial fluid analysis or imaging techniques that are not available in most centers, leaving healthcare professionals to rely only on clinical presentations and their experience. In addition, gout management requires the evaluation of multiple aspects, such as monitoring of serum uric acid (sUA) level (which should be reduced to <6 mg/dL) to ensure adherence and efficacy of treatment, evaluation of patient's risk profile and comorbidities, and continuous assessments to manage clinical manifestations. An important premise in gout management is non-pharmacological intervention; however, pharmacological urate-lowering therapy is crucial for an optimal control of the disease. Available options include xanthine-oxidase inhibitors (XOI), targeting uric acid overproduction, and uricosuric agents which target the predominant cause of hyperuricemia (under-excretion). Among these, lesinurad is the novel uricosuric agent to be used in combination with XOI in patients with gout not adequately controlled with XOI alone, which can further contribute to the control of hyperuricemia in gout. CONCLUSIONS: Multidisciplinary management is crucial for the diagnosis and treatment of gout, in order to ensure treatment continuity and improve management. This review, therefore, advises that educational activities for General Practitioners and specialists should be implemented to help raise awareness on gout diagnosis, monitoring, and treatment.

A rare case of atypical chronic lymphocytic leukaemia presenting as nephrotic syndrome.

Chronic lymphocytic leukaemia (CLL) is characterised by a lymphocytosis of mature-appearing clonal CD5+, CD23+ B lymphocytes. CLL cells arise from the bone marrow and infiltrate lymphoid tissues such as lymph nodes and spleen. Presentation is usually through discovery of lymphocytosis or lymphadenopathy. Unusual presentations, especially paraneoplastic syndromes are rare. Here, we describe a rare case presenting with severe nephrotic syndrome associated with the presence of a monoclonal protein in serum. Workup for suspected plasma cell dyscrasia led instead to the diagnosis of bone marrow infiltration by atypical CLL without lymphocytosis. Renal biopsy showed a glomerulonephritis that turned out to be paraneoplastic as it went into remission after treatment for CLL. Our case shows an unusual presentation of CLL and prompts for increased awareness of lymphoproliferative disorders in the context of seemingly unrelated conditions that may be paraneoplastic in origin.

Systemic lupus erythematosus and HIV infection: a whimsical relationship. Reports of two cases and review of the literature.

UNLABELLED: Systemic lupus erythematosus (SLE) is rarely reported in association with HIV infection. We describe two unpredictable cases and provide a review of the literature. Retrospective analysis of the medical records of two HIV-infected patients diagnosed with SLE and admitted at Luigi Sacco Hospital (Milano, Italy). Search of the literature from 1981 to 2012 and review of the cases reported. Case 1: a 32-year-old HIV-infected African woman who developed a SLE flare after re-introduction of antiretroviral therapy (ART). The flare was characterized by bullous skin eruption and membranous glomerulonephritis. Case 2: a 44-year-old Caucasian woman, admitted to our hospital because of lacunar stroke: HIV infection and SLE were simultaneously diagnosed. LITERATURE: 55 cases of SLE in the setting of HIV infection were reported. Forty-five patients met the requirements of the American College of Rheumatology for the diagnosis of SLE. The diagnosis of SLE preceded HIV infection in six patients. On the contrary, in 29 patients, HIV infection was reported before SLE. Median CD4+ count at SLE diagnosis was 361 cells/µl. A SLE manifestation following ART immune recovery was documented in 18.2% of the cases. On the contrary, the progression of HIV infection paralleled with SLE remission in 22.5% of the patients. The study shows that an autoimmune disease such as SLE can occur despite the loss of immunocompetence caused by HIV infection. Moreover, SLE and HIV infection influence each other possibly through immunologic mechanisms determining awkward manifestations.

Lung ultrasonography for the assessment of rapid extravascular water variation: evidence from hemodialysis patients.

Chest ultrasonography is a useful tool to assess extravascular lung water at bedside. In presence of interstitial-alveolar imbibition, vertical artifacts arising from the pleura are detected; these are called B-lines. Although a positive linear correlation between B-lines and extravascular lung water has been shown in symptomatic heart failure patients, the subclinical phase of pulmonary imbibition and the clearance of B-lines after rapid body fluid removal have been less investigated. The aim of this study was to assess if chest ultrasound could detect lung water imbibition and its variations induced by dialysis, an experimental model of controlled rapid fluid loss. Forty-one patients undergoing hemodialysis were studied. Total number of B-lines from ultrasound chest scanning and vena cava diameters were measured before and after treatment. Before dialysis, most of the patients presented ultrasound signs of pulmonary imbibition despite the absence of dyspnea; the number of B-lines was associated with the accumulated weight before treatment (p < 0.05) as well as with the residual weight after dialysis (p < 0.01); B-lines and end-inspiratory and end-expiratory vena cava diameters were also significantly reduced after dialysis. Moreover, B-lines reduction was significantly related to weight loss. Ultrasound performed at the bedside can detect lung water and intravascular overload and their reduction after dialysis in yet asymptomatic patients. These observations add further evidence regarding the use of lung ultrasound and inferior vena cava measurement in estimating volume overload and monitoring the response to therapy both in hemodialysis and congestive heart failure patients.

Pancake polycystic kidney: case report.

Pancake kidney is a very rare fusion abnormality, characterised by the presence of a renal parenchymal mass located in pelvic site, generally with two pelvies and two ureters and without an intervening fibrous septum. The case here reported describes a condition of "pancake kidney", eventually associated with polycystic disease and abnormous vascular supply. Hypertension and microscopic hematuria were the only clinical signs.

Fibrillary glomerulonephritis with prevalent IgA deposition associated with undifferentiated connective tissue disease: A case report.

We described a 41-year-old female patient, who presented with proteinuria occurring 5 years after the onset of an undifferentiated connective tissue disease (UCTD). At renal biopsy, a pattern of focal necrotizing glomerulonephritis with mesangial and parietal deposition of the IgA, C3 and K chains was observed. Electron microscopy showed organized fibrillary deposits in mesangial, subendothelial, intramembranous and subepithelial sites. Fibrils were randomly arranged, had no hollow core and had a diameter ranging between 10 and 23 nm. This case showed a rare combination of fibrillary glomerulonephritis and prevalent IgA deposition, in the clinical context of UCTD.

Acetylcysteine and non-ionic isosmolar contrast-induced nephropathy--a randomized controlled study.

INTRODUCTION: Intravenous administration of saline and non-ionic isosmolar contrast media significantly reduces the incidence of contrast-induced nephropathy, one of the most common causes of acute renal failure. Results with oral N-acetylcysteine are conflicting. The aim of our study was to evaluate the prophylactic role of N-acetylcysteine in patients with stable chronic renal failure undergoing coronary and/or peripheral angiography and/or angioplasty. METHODS: We randomized 200 elective, consecutive patients (mean age 74.9 +/- 7.3 years; 65% male, 25% diabetics) with basal creatinine clearance 0.5 mg/dl or >25% within 3 days after the procedure. Serum creatinine was measured at baseline, 24, 48 and 72 h after the procedure. RESULTS: Contrast-induced nephropathy was 8/99 (8.1%) in the N-acetylcysteine group versus 6/101 (5.9%) in the placebo group, P = 0.6. No difference was noted in high-risk subgroups such as diabetics (4/25 versus 2/25 P = 0.4) and those with serum creatinine clearance <42.3 ml/min (5/54 versus 4/48; P = 0.9). CONCLUSION: In our experience, N-acetylcysteine did not prevent contrast-induced nephropathy in patients receiving isosmolar (iodixanol) contrast media and adequate hydration.