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Manole Cojocaru, Inimioara Cojocaru, Isabela Silosi and Camelia Vrabie

Autoimmune Lymphoproliferative Syndrome

The autoimmune lymphoproliferative syndrome (ALPS) is a rare disease. ALPS is an inherited condition that affects both sexes. ALPS is not cancer, it is not infectious, and its incidence has not yet been estimated. ALPS generally does not lead to death and most individuals with ALPS are able to live normal lives. ALPS is a disorder associated with abnormal lymphocyte apoptosis, lymphoproliferation, and autoimmunity. Serologic testing is critical in the evaluation of these individuals. Lymphoproliferation in ALPS patients is generally benign, but they are at increased risk for the development of Hodgkin's and non-Hodgkin's lymphoma. It is characterized by massive lymphoadenopathy, splenomegaly, autoimmunity including episodes of immune hemolityc anemia, thrombocytopenia, and neutropenia. ALPS patients have lymphocytosis and a number of lymphocyte abnormalities, including the marked expansion of T lymphocytes that express alpha/beta T-cell receptors, but neither CD4 nor CD8 surface markers (TCR alpha/beta+; CD4-; CD8- cells).

Open access

Inimioara Cojocaru, Manole Cojocaru, Isabela Silosi and Camelia Vrabie

Central Nervous System Manifestations in Rheumatic Diseases

Patients with multi-system rheumatic conditions may have a disease affecting the central nervous system (CNS). Central nervous system manifestations vary according to the location of the lesion and range from focal findings (e.g., stroke-like presentations), although serious neurological complications in rheumatic disease appear to be rare. The most prominent features of neurological involvement in rheumatic diseases include cerebral ischaemia and psychiatric symptoms. Little information is available on the prevalence of neurological disease in patients with a rheumatological diagnosis. Involvement of the CNS may be a striking early or presenting feature with a wide variety of manifestations. There is more clarity about the CNS syndromes attributable to systemic lupus erythematosus and new insights into the central mechanisms involved in the manifestations of Sjögren's syndrome and rheumatoid arthritis. Severe CNS involvement is associated with poor prognosis, and high mortality rate. We review the spectrum of neurological diseases in patients with a rheumatological diagnosis.

Open access

Manole Cojocaru, Inimioara Cojocaru, Isabela Silosi and Camelia Vrabie

Kidney Damage in Autoimmune Diseases

Renal involvement in autoimmunity has many facets. Glomerular, tubular and vascular structures are targeted and damaged as a consequence of autoimmune processes. Immunologically mediated kidney diseases represent the third most common cause of end-stage renal failure (after diabetic and hypertensive nephropathies). Appropriate evalution of patients with immune-mediated kidney diseases requires a meticulous history and physical examination, with particular attention to the urinalysis, tests of renal function and often renal biopsy. The thorough clinician should personally review microscopic urinalysis in any case in which there is a reasonable index of suspicion of immune-mediated renal disease. In this article we propose to highlight recent developments, with particular reference to renal autoimmunity. Systemic lupus erythe-matosus affects many parts of the body: primarily the skin and joints, but also the kidneys. Goodpasture's syndrome involves an autoantibody that specifically targets the kidneys and the lungs. IgA nephropathy is a form of glomerular disease that results when immunoglobulin A (IgA) forms deposits in the glomeruli, where it creates inflammation. Future research could look for how the disease occurs, and how to easily test for its presence so that early treatment could be started.