Duration of Disease and Long-Term Outcomes in Patients With Difficult-To-Treat Recurrent Pericarditis: A Chronic Condition Treated With Nonsteroidal Anti-Inflammatory Drugs, Colchicine, Corticosteroids, and Anti-Interleukin-1 Agents.

Objective: We aimed to investigate the remission rate and disease duration in idiopathic or post-cardiac injury pericarditis and risk factors for disease duration and anti-interleukin-1 (IL-1) agent discontinuation.

Methods: This was a multicenter, longitudinal, observational study including 370 patients (51.4% female).

Results of a nation-wide cohort study suggest favorable long-term outcomes of clone-targeted chemotherapy in immunotactoid glomerulopathy.

Immunotactoid glomerulopathy is a rare disease defined by glomerular microtubular immunoglobulin deposits. Since management and long-term outcomes remain poorly described, we retrospectively analyzed results of 27 adults from 21 departments of nephrology in France accrued over 19 years. Inclusion criteria were presence of glomerular Congo red-negative monotypic immunoglobulin deposits with ultrastructural microtubular organization, without evidence for cryoglobulinemic glomerulonephritis.

[Pulmonary and renal involvement in a TNFα antagonist drug-induced sarcoidosis].

Several cases of sarcoidosis with pulmonary, neurological or ophthalmological involvement occurring during a treatment by anti-TNFα have been reported in the literature. We report a 66-year-old man who presented with renal failure and hypercalcaemia, associated with fatigue, shortness of breath and dry cough. He was receiving infliximab since 2004 for psoriatic arthritis.

CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients.

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, with noncodified therapeutic management and high mortality. No treatment has yet been shown to improve survival in these patients. We conducted a multicenter prospective observational cohort study to assess whether extraskeletal manifestations and interferon-α treatment would influence survival in a large cohort of ECD patients.

Immunoglobulin deposition disease with a membranous pattern and a circulating monoclonal immunoglobulin G with charge-dependent aggregation properties.

A 62-year-old woman presented with nephrotic syndrome, monoclonal gammopathy, and membranous-like nephropathy with nonorganized deposits composed of monoisotypic immunoglobulin G1 lambda protein. Nephrotic syndrome remitted after a brief course of treatment with melphalan despite ongoing production of the monoclonal protein. The circulating monoclonal immunoglobulin G1 lambda showed unusual in vitro aggregation properties, including dependence on low ionic strength and neutral pH, suggesting that electrostatic interactions had a role in the precipitation process.

[Acute renal failure following a treatment with acyclovir].

Unlabelled: The antiviral molecule acyclovir can be responsible of severe renal dysfunction. Intratubular crystal precipitation of the drug may represent a major pathogenetic mechanism.

Observation: A 30-year old, immunocompetent woman was admitted in the neurology unit for a viral meningo-encephalitic syndrome.

A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis.

Objective: To investigate the effectiveness and side effects of oral versus pulse cyclophosphamide (CYC) in combination with corticosteroids (CS) in the treatment of systemic Wegener's granulomatosis (WG).

Methods: Patients with newly diagnosed systemic WG were enrolled in a prospective, randomized trial. At the time of diagnosis, prior to randomization, every patient received a daily injection of methylprednisolone for 3 days, followed by daily oral prednisone (1 mg/kg/day) and a 0.

Caffeine clearance in subjects with constitutional unconjugated hyperbilirubinemia.

To investigate the behaviour of caffeine (CAF) in patients with Gilbert's Syndrome (GS), a combined oral loading test of caffeine and chenodeoxycholic acid was performed in 14 healthy subjects and 71 patients with GS. Indocyanine green (ICG) kinetics was tested in 50 subjects with GS and in all control subjects. Fasting serum bile acids (SBA) and clearance after CDCA loading were within normal range in normal and GS subjects.

Increased risk of Pneumocystis carinii pneumonia in patients with Wegener's granulomatosis.

Combining cyclophosphamide (Cy) and corticosteroids has dramatically improved the prognosis of Wegener's granulomatosis (WG). But this treatment carries the risks of severe infectious complications and drug toxicity. During a 10-month period, we observed 6 cases of Pneumocystis carinii pneumonia (PCP) in 23 patients with biopsy-proven WG and renal involvement.

["Idiopathic" extracapillary glomerulonephritides without immune deposits are vascularitides: clinical and serologic analysis].

To determine the spectrum of systemic diseases potentially associated with pauci-immune rapidly progressive glomerulonephritis (GN), most of which being considered as idiopathic, we have analyzed extra-renal manifestations, occurrence of extra-glomerular vasculitis and incidence and specificity of ANCAs in 40 patients selected only on histological criteria. Extra-renal symptoms were unexpectedly observed in all patients but one, and were suggestive of vasculitis in 24 of them. Extra-glomerular vasculitis was evidenced in 18 kidney biopsies and four biopsies from other organs.

Necrotizing crescentic glomerulonephritis without significant immune deposits: a clinical and serological study.

To determine the spectrum of systemic diseases associated with pauci-immune necrotizing crescentic glomerulonephritis, we have analysed extra-renal manifestations, occurrence of extra-glomerular vasculitis and incidence and specificity of antinuclear cytoplasmic antibodies (ANCA) in 40 patients selected only on renal histological criteria. Extra-renal symptoms were unexpectedly observed in all patients but one, and were suggestive of vasculitis in 24. Extra-glomerular vasculitis was seen in 18 kidney biopsies and four biopsies from other organs.

[Extracapillary and necrotizing glomerulonephritis without immunoglobulin deposits: clinical presentation, prognostic factors and response to treatment. Apropos of 42 cases].

Forty-two cases of pauci-immune necrotizing glomerulonephritis were reviewed on a 10 years period. Selection was exclusively based on histological criteria, i.e.

[Necrotizing extracapillary glomerulonephritis without immunoglobulin deposits. Renal localization of systemic vasculitis with anti-cytoplasm polynuclear antibodies].

Necrotizing and crescentic glomerulonephritis without immunoglobulin deposits (also called pauci-immune glomerulonephritis) is the cause of approximately 50 percent of acute renal failures of glomerular origin. Our study, based on 40 case-records selected on histological criteria, and on data from the literature, shows that in most cases, if not all, this type of glomerulonephritis is part of a wider systemic vasculitis which predominantly affects the glomerular capillaries. Anti-neutrophil cytoplasm antibodies are detected in three-quarters of the cases, with a specific distribution that varies according to the clinical features.

Delayed extensive arterial dissection after percutaneous transluminal angioplasty for transplant renal artery stenosis.

A 26-year-old renal transplant recipient with graft artery stenosis underwent percutaneous transluminal angioplasty (PTA), which was followed by extensive renal artery dissection leading to graft loss. This report reminds one that, although widely considered a safe procedure, PTA is not free of complications.

Modifications in the serum concentrations of prolyl hydroxylase in patients with chronic hepatitis B during and after interferon therapy.

A total of eight patients with chronic active HBsAg-positive hepatitis was treated with recombinant interferon-alpha 2b for 12 months and serum aspartate aminotransferase, alanine aminotransferase, gamma-globulin and prolyl hydroxylase concentrations were determined every 3 months. Liver biopsies after 12 months' treatment revealed a significant (P less than 0.05) reduction in the histological activity score.

Destructive spondyloarthropathy in dialysis patients: an overview.

Destructive spondyloarthropathy has been increasingly recognized in dialysis patients. We have reviewed 39 observations published in the literature and added 11 personal cases in order to define the clinical and radiological presentation of the disease. The many hypotheses that have been proposed for its pathogenesis are discussed, including secondary hyperparathyroidism, microcrystal deposition, beta 2-microglobulin-associated amyloidosis and aluminum intoxication.

Early and late occurrences of destructive spondyloarthropathy in haemodialysed patients.

In a series of 100 patients dialysed for 3 months to 17 years destructive spondyloarthropathy was diagnosed in 11 cases. Lower cervical spine was involved in ten and dorsal spine in one. When compared to patients without arthropathy, spinal involvement was associated with longer duration of dialysis and greater prevalence of parathyroidectomy.