Subject(s)
Edema/genetics , Mutation, Missense , Point Mutation , Receptors, Tumor Necrosis Factor, Type I/genetics , C-Reactive Protein/analysis , Edema/blood , Etanercept , Extremities , Face , Humans , Immunoglobulin G/therapeutic use , Leukocyte Count , Male , Middle Aged , Phenotype , Receptors, Tumor Necrosis Factor/therapeutic use , Receptors, Tumor Necrosis Factor, Type I/antagonists & inhibitors , Receptors, Tumor Necrosis Factor, Type I/physiology , Recurrence , SyndromeABSTRACT
The lupus anticoagulant-hypoprothrombinemia syndrome (LAHS)--the association of acquired factor II deficiency and lupus anticoagulant--is a rare disease drastically different from antiphospholipid syndrome in that it may cause predisposition not only to thrombosis but also to severe bleeding. We performed a retrospective study of 8 patients with LAHS referred to 6 French tertiary care centers between January 2003 and February 2011, and a literature review retrieving all related articles published between 1960 and April 2011. Including our 8 new cases, LAHS has been reported in 74 cases. The disease mostly occurs in young adults, with a female to male sex ratio of 1.4. Associated conditions mostly include autoimmune diseases such as systemic lupus erythematosus and infectious diseases. Bleeding is a frequent feature (89% of cases), while arterial and/or venous thrombosis is less common (13%). Factor II level is severely decreased at diagnosis (median value, 11%; range, 1%-40%). LAHS associated with autoimmune diseases is more persistent than LAHS associated with infection, and hemorrhagic complications are more common. Corticosteroids should be considered the first-line treatment, but the thrombotic risk strongly increases during treatment because of the improvement of factor II level. Despite the fact that 50% of patients develop severe bleeding, the mortality rate is <5%, after a median follow-up of 13 months (range, 0.5-252 mo). LAHS associated with autoimmune diseases should be diagnosed and managed carefully because the disease is persistent and severe hemorrhagic complications are common.
Subject(s)
Antiphospholipid Syndrome/complications , Hypoprothrombinemias/complications , Lupus Coagulation Inhibitor , Lupus Erythematosus, Systemic/complications , Prothrombin/immunology , Adolescent , Adrenal Cortex Hormones , Adult , Aged , Antiphospholipid Syndrome/diagnosis , Autoimmune Diseases/complications , Female , France , Humans , Hypoprothrombinemias/diagnosis , Hypoprothrombinemias/immunology , Lupus Erythematosus, Systemic/diagnosis , Male , Middle Aged , Retrospective StudiesABSTRACT
Chronic graft-versus-host disease (GVHD) sometimes mimics autoimmune diseases. We report the case of a 39-year-old patient who presented atypical polymyositis without elevated creatinine phosphokinase, related to a chronic GVHD following interruption of immunosuppressive treatment. Treatment with cyclosporine and corticosteroids resulted in complete and sustained remission of the polymyositis. The symptoms of chronic GVHD-related polymyositis are indistinguishable from those of idiopathic polymyositis. The context of transplantation and a decrease or interruption of prophylaxis suggest the diagnosis of GVHD-related polymyositis, especially if other manifestations of GVHD are associated. A suitably adapted treatment (association of corticotherapy and cyclosporine) improves polymyositis, and in most cases, a normal clinical state is achieved even if the symptoms were severe.
Subject(s)
Graft vs Host Disease/complications , Polymyositis/etiology , Adrenal Cortex Hormones/therapeutic use , Adult , Cyclosporine/therapeutic use , Humans , Immunosuppressive Agents/therapeutic use , Male , Polymyositis/drug therapy , Polymyositis/immunologyABSTRACT
Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection of the central nervous system with JC virus. Few cases have been described in lupus patients. We describe biopsy-proven PML in a lupus patient receiving mycophenolate mofetil and corticosteroids. Although the patient received no antiviral treatment, the polymerase chain reaction test for JC virus became negative in cerebrospinal fluid after immunosuppressant discontinuation and the patient survived. We discuss the restoration of immune efficiency after immunosuppressant discontinuation in this case and compare the clinical, radiological and histological features with the inflammatory PML form described in human immunodeficiency virus-infected patients.
Subject(s)
Immune System/immunology , Immunosuppressive Agents/adverse effects , Leukoencephalopathy, Progressive Multifocal/immunology , Lupus Erythematosus, Systemic/drug therapy , Recovery of Function/immunology , Withholding Treatment , Adrenal Cortex Hormones/administration & dosage , Adrenal Cortex Hormones/adverse effects , Brain/immunology , Brain/physiopathology , Brain/virology , Female , Humans , Immune Reconstitution Inflammatory Syndrome/immunology , Immune Reconstitution Inflammatory Syndrome/physiopathology , Immune Reconstitution Inflammatory Syndrome/prevention & control , Immune System/drug effects , Immunocompromised Host/drug effects , Immunocompromised Host/immunology , Immunosuppressive Agents/administration & dosage , JC Virus/drug effects , JC Virus/immunology , Leukoencephalopathy, Progressive Multifocal/physiopathology , Leukoencephalopathy, Progressive Multifocal/virology , Middle Aged , Mycophenolic Acid/administration & dosage , Mycophenolic Acid/adverse effects , Mycophenolic Acid/analogs & derivatives , Recovery of Function/drug effectsABSTRACT
Therapeutic management of blood hypereosinophilia depends on its underlying cause. The cause may be clearly established (parasitic infestation) or more hypothetical (systemic disease). Blood eosinophils often return to normal levels after treatment of either the cause or the associated eosinophilic disease. A targeted approach is more difficult when the cause is unknown (unexplained chronic hypereosinophilia). Various conventional treatments (corticosteroids, hydroxyurea, interferon) have been somewhat effective. The identification of new cellular and molecular markers with diagnostic and pathophysiologic significance makes more rational approaches possible.