[Clinical, ultrasound and treatment of aneurysms and pseudoaneurysms in hemodialysis vascular access]. / Clinica, ecografia e trattamento degli aneurismi e pseudoaneurismi nell'accesso vascolare dell'emodializzato.

Aneurysms (AN) and pseudoaneurysms are among the complications of vascular access. AN is a focal area of expansion, concentric or eccentric, with the wall consistency the same as all elements of the vessel wall (intima, media and adventitia). Pseudoaneurysm, or false aneurysm, is a blood harvesting without vascular wall, it is characterized by a reactive capsule of connective tissue that delimits it. The K/DOQI guidelines recommend a regular program of monitoring and surveillance of the vascular access. Color-Doppler ultrasound is considered a valuable tool in the preoperative evaluation and in the follow-up. The echo-color-Doppler surveillance plays an important role in diagnosis of aneurysm. It allows monitoring the evolution of the aneurysm, studying vessels walls, thickened because of intimal hyperplasia and to identify the presence of thrombotic material and/or calcification of the wall. Early identification of complications and the adoption of corrective measures will extend the life of the vascular access, with benefit for the patient. Moreover, it will reduce health care costs.

Renal involvement in anti-neutrophil cytoplasmic autoantibody associated vasculitis.

Renal involvement is a common and often severe complication of anti-neutrophil cytoplasmic autoantibody (ANCA) associated vasculitides (AAV). With the exception of Churg-Strauss syndrome (CSS), where kidney involvement is not a prominent feature, renal disease is present in about 70% of patients with Wegener's granulomatosis, now called granulomatosis with polyangiitis (GPA) and in almost 100% of patients with microscopic polyangiitis (MPA). Kidney involvement is generally characterized by a pauci-immune necrotizing and crescentic glomerulonephritis with a very rapid decline of renal function (rapidly progressive glomerulonephritis). Even though there are not qualitative differences in glomerular lesions in patients with GPA or with MPA, chronic damage is significantly higher in MPA (and/or P-ANCA positive patients) than in GPA (and/or C-ANCA positive patients). If untreated necrotizing and crescentic glomerulonephritis has an unfavorable course leading in a few weeks or months to end stage renal disease. Serum creatinine at diagnosis, sclerotic lesions and the number of normal glomeruli at kidney biopsy are the best predictors of renal outcome. Corticosteroids and cyclophosphamide (with the addition of plasma exchange in the most severe cases) are the cornerstone of induction treatment of ANCA-associated renal vasculitis, followed by azathioprine for maintenance. Rituximab is as effective as cyclophosphamide in inducing remission in AAV and probably superior to cyclophosphamide in patients with severe flare, and could be preferred in younger patients in order to preserve fertility and in patients with serious relapses.

Azathioprine hypersensitivity: report of two cases and review of the literature.

Azathioprine (AZA) is a widely-used drug in the treatment of different diseases such as vasculitis, systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel diseases and in renal transplantation. Side effects of AZA can be classified as toxic, mainly dose related (myelosuppression and hepatotoxicity) and idiosyncratic, mainly dose independent. While the toxic effects are common and well documented, the hypersensitivity reactions are rare and it is not often easy to distinguish them from systemic sepsis or disease recurrence. We report two cases of AZA hypersensitivity occurring in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis each mimicking a vasculitis relapse or a septic complication of immunosuppression, as well as a review of the literature.

The use of laboratory tests in diagnosis and monitoring of systemic lupus erythematosus.

Clinical immunology laboratories play an essential role in diagnosis and monitoring of systemic lupus erythematosus (SLE). To obtain the best results in terms of diagnostic performance and clinical usefulness, the following recommendations should be fulfilled: Indirect immunofluorescence on Hep-2 cells remains the method of choice for the detection of anti-nuclear antibodies (ANA). The sensitivity of ANA test for SLE is very high (almost 100%) but its specificity low since ANA can be present in a number of different clinical conditions and even in normal controls. Anti-dsDNA antibodies are highly specific for SLE and present in a high proportion of SLE patients (40-80%). The method of choice for anti-ds DNA is the Farr assay; however, the necessity of using radioactive material decreases its applicability. As an alternative, immunofluorescence on Crithidia Luciliae can be used in the diagnostic phase for its high specificity. It is not advisable to use ELISA, in the diagnostic phase, due to its low specificity. The quantitative determination of anti-dsDNA is useful for monitoring patients, in particular in the presence of nephritis. For monitoring, a quantitative method should be used (Farr assay or ELISA). The detection of antibodies to extractable nuclear antigens (ENA) and to phospholipids (Lupus anticoagulant and anti-cardiolipin antibodies with a beta2 glycoprotein I-dependent method) are useful to identify subgroups of patients at risk for some clinical manifestations (i.e. anti-phospholipid syndrome). New assays (anti-C1q and anti-nucleosome antibodies) have been recently proposed for diagnosis (anti-nucleosome) and monitoring SLE patients (anti-C1q and anti-nucleosome antibodies), with promising results. Among biological parameters, urinary levels of monocyte chemoattranct protein 1 (MCP1) seem to be the most useful to monitor nephritis activity in lupus patients.