The expanding spectrum of renal diseases associated with antiphospholipid syndrome.

Background: The association of thrombotic events and/or pregnancy complications with circulating antiphospholipid antibodies defines antiphospholipid syndrome (APS). In previous reports, renal involvement in APS consisted mainly of thrombotic vascular complications involving large vessels or intrarenal small-sized vessels (APS nephropathy). We report 9 cases of glomerulonephritis associated with APS.

[Interferon-alpha and auto-immunity].

Purpose: To assess the role of endogenous interferon alpha (IFN) in auto-immune experimental models and human diseases, and to evaluate its iatrogenic potential as a therapeutic agent.

Main Points: IFN is a cytokine involved in cellular immunity, that promotes both differentiation of dendritic cells and the TH1 pathway. Auto-immune side-effects of recombinant IFN depend on IFN dosage and the pathology concerned.

Magnetic resonance imaging criteria for distinguishing between inclusion body myositis and polymyositis.

Objective: To develop diagnostic imaging criteria for polymyositis (PM) and sporadic inclusion body myositis (sIBM).

Methods: We investigated 220 patients with suspected inflammatory myopathies by magnetic resonance imaging (MRI). Findings were compared with the results of clinical and biological examinations and muscle biopsy.

Pure red-cell aplasia and antierythropoietin antibodies in patients treated with recombinant erythropoietin.

Background: Within a period of three years, we identified 13 patients in whom pure red-cell aplasia developed during treatment with recombinant human erythropoietin (epoetin). We investigated whether there was an immunologic basis for the anemia in these patients.

Methods: Serum samples from the 13 patients with pure red-cell aplasia were tested for neutralizing antibodies that could inhibit erythroid-colony formation by normal bone marrow cells in vitro.

Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases.

Objective: To evaluate the response to treatment in a large cohort of patients with systemic lupus erythematosus (SLE) associated with autoimmune thrombocytopenia.

Methods: Response to treatment was assessed retrospectively in 59 patients with SLE, either definite (n = 44) or incomplete (n = 15), associated with frank autoimmune thrombocytopenia (defined as platelet count < 50 x 10(9)/l). Response to treatment was classified as complete (CR: platelet count > 150 x 10(9)/l), partial (PR: platelet count > 50 x 10/l), or failure (FR) in the other cases.

Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center.

Dermatologic manifestations of relapsing polychondritis (RP) have been relatively poorly studied compared to other manifestations. In this study we describe dermatologic manifestations in a large series of patients with RP and the corresponding pathologic findings. In this retrospective, single-center review of 200 patients diagnosed with RP according to Michet's criteria, we analyzed separately those suffering from associated diseases with potential dermatologic involvement or chronic dermatitis.

[Fainting after drinking a glass of whiskey-soda...].

Background: Alcohol intolerance is a rare syndrome that usually involves pain, pruritus, or vasomotor phenomena consecutive to moderate alcohol intake. This syndrome may unmask various malignancies, especially Hodgkin's disease or non-Hodgkin lymphoma, and carcinoma of the cervix.

Case Report: A patient who became unconscious after drinking a glass of whiskey-cola was explored for alcohol intolerance.

A novel missense mutation (C30S) in the gene encoding tumor necrosis factor receptor 1 linked to autosomal-dominant recurrent fever with localized myositis in a French family.

Objective: To characterize both phenotypic (clinical features and magnetic resonance imaging [MRI] findings) and genotypic aspects of autosomal-dominant recurrent fever, also known as tumor necrosis factor receptor (TNFR)-associated periodic syndrome (TRAPS), in a French family and to investigate the role of the mutated 55-kd tumor necrosis factor alpha (TNFalpha) receptor (TNFR1) in the pathogenesis of the disease.

Methods: The coding region of TNFR1 was sequenced in 2 individuals with TRAPS (the propositus and her grandfather) and in 3 clinically unaffected relatives. Expression of soluble TNFR1 (sTNFR1) was investigated in 3 of the family members carrying a C30S mutation in TNFR1, and was compared with the levels of soluble TNFR2 (sTNFR2) by enzyme-linked immunosorbent assay.

Systemic vasculitis with bilateral perirenal haemorrhage in chronic myelomonocytic leukaemia.

The cases of two patients with chronic myelomonocytic leukaemia associated with periarteritis nodosa-like, antineutrophil cytoplasmic antibody negative, systemic vasculitis, are reported. A 61 year old man was admitted with fever, diffuse myalgia, and abdominal pain. Blood and bone marrow examination showed chronic myelomonocytic leukaemia.

[Retinopathy in acute pancreatitis].

Acute pancreatitis may involve remote organ systems, including the eye. Funduscopy may reveal in these rare cases presence of a typical ischemic retinopathy, similar to that diagnosed after severe traumas, known as the Purtscher's retinopathy. Pathogenesis of retinopathy in alcoholic retinopathy remains controversial: occurrence of fat embolism or leucothrombosis is discussed in the literature.

[Polyvalent intravenous immunoglobulins in systemic lupus].

Purpose: To evaluate intravenous immunoglobulins (IVIG) treatment, which is immunomodulatory but not immunosuppressive, in SLE.

Main Issues: IVIG indications in SLE could be categorized as already validated as for chronic polyradiculoneuropathy or thrombocytopenia; failure of classical treatment in threatening active disease; undetermined manifestation as reactive hemophagocytic syndrome which could be both disease-specific or iatrogenic, infection-related. To date, no published study has firmly established the efficacy of IVIG in SLE.

Acute posterior multifocal placoid pigment epitheliopathy associated with Wegener's granulomatosis.

Purpose: To report acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in the course of systemic Wegener's granulomatosis.

Methods: A complete ophthalmologic evaluation was undertaken, including fluorescein and indocyanine green angiography.

Results: Two patients diagnosed with Wegener's granulomatosis developed multiple white-yellow subretinal areas with smooth margins typical of APMPPE.

Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients.

Sneddon syndrome is characterized by the association of livedo reticularis and cerebral ischemic arterial events (stroke or transient ischemic attack). Reported prevalence of antiphospholipid antibodies is highly variable. We conducted this study to compare the clinical and pathologic features of patients with Sneddon syndrome according to the presence or absence of antiphospholipid antibodies.

Renal involvement in systemic lupus erythematosus. A study of 180 patients from a single center.

Charts of 180 patients (147 women, 33 men) with systemic lupus erythematosus (SLE) complicated by renal involvement were retrospectively analyzed from a series of 436 patients. Mean age at renal disease onset was 27 years. Thirty-six percent of the patients had renal involvement after diagnosis of lupus, for 30.

The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus.

We address the relationship between reactive hemophagocytic syndrome (RHS), systemic lupus erythematosus (SLE) activity, and treatment in 4 female patients with SLE. Febrile pancytopenia was related to cytologically proven RHS in all patients. Followup was 45+/-7 months from RHS onset.