Autoimmune Collagen Vascular Diseases Research Articles

“What Lies Beneath” – Macrophage Activation Syndrome Unveiled Systemic Lupus Erythematosus

Macrophage activation syndrome (MAS) is a potentially fatal condition characterized by massive systemic inflammatory response in the setting of a myriad of systemic disorders such as autoimmune collagen vascular disease, infections, drug-induced, and malignancies. It falls under a group of disorders known as hemophagocytic lymphohistiocytosis of which familial and acquired types have been described. Here, we report a case of acquired MAS secondary to acute systemic lupus erythematosus (SLE). Early diagnosis and timely intervention resulted in a favorable outcome.

Indian Dermatologists Wield Technology to Combat COVID-19!

Indian Dermatologists Wield Technology to Combat COVID-19!

Systemic lupus erythematosus and overlap: A clinician perspective

Autoimmune inflammatory rheumatic diseases, also termed as autoimmune collagen vascular diseases, as the name suggests are the group of illness arising from a combination of loss of tolerance to self-antigens and shifting of the immune system into self-destructive overdrive. These are commonly classified under six mutually exclusive diseases which include systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis, polymyositis, rheumatoid arthritis (RA), and primary Sjogren syndrome, the so-called definitive connective tissue diseases. Undifferentiated and mixed connective tissue disease and overlap syndrome have also been added to the classification. An initially undifferentiated connective tissue disease may later evolve into a differentiated connective tissue disease. Here, we define the various connective tissue diseases and elaborate more upon SLE and its overlap, including the overlap of RA (RHUPUS).

Autoimmune collagen vascular diseases: Kids are not just little people

Morphea, dermatomyositis (DM), and discoid lupus erythematosus (DLE) are autoimmune collagen vascular diseases that can present at any age. In all three of these diseases, the tenants of diagnosis and treatment are largely the same in both children and adults, with a few notable differences. Children with morphea are more likely to present with the linear subtype and have a higher incidence of extracutaneous manifestations. Children often need early aggressive systemic treatment to try to prevent long-term sequelae of morphea. In DM, adult disease has a clear association with malignancy that is not seen in children. Adults have a higher rate of pulmonary involvement and increased mortality, whereas calcinosis is more common in juvenile DM. DLE in adults is generally considered to have a low rate of progression from discoid lesions alone to systemic lupus erythematosus (SLE). DLE is less common in children, but several studies have suggested a higher rate of progression from DLE to SLE in children compared with adults.

Juggling Multiple Guidelines: A Woman's Heart in the Balance.

In 2011, the American Heart Association (AHA) issued the pivotal "Effectiveness-based Guidelines for the Prevention of Cardiovascular Disease in Women-2011 Update." In the interim, multiple guidelines have dramatically altered recommendations for preventive cardiovascular care. This article addresses how I juggle these multiple guidelines in my clinical practice. In brief, my approach to risk stratification is to use the Pooled Cohort Equations, but I also routinely assess the risk factors unique to or predominant in women such as pregnancy complications and systemic autoimmune collagen vascular diseases. I follow the 2013 AHA/American College of Cardiology (ACC) Guidelines on Lifestyle Management to Reduce Cardiovascular Risk, but find value in the detailed aspects of physical activity recommendation in the 2011 Women's Guideline, including those for weight loss or weight loss maintenance. Based solely on epidemiological data, I consider a blood pressure (BP) of 120//80 mmHg ideal in women who remain asymptomatic at that level. I typically titrate BP therapy to 120-130/80-90 mmHg as tolerated. I endorse the current ACC/AHA recommendations for cholesterol management, but for my women patients older than age 75 who previously tolerated a high-intensity statin, I continue that medication or whatever statin they tolerated through age 75. For women older than age 75 with a recent acute atherosclerotic cardiovascular disease (ASCVD) event, a high-risk population, I follow the guideline for younger patients. As ASCVD events are becoming more common before 40 years of age, I screen younger women earlier when risk factors unique to or predominant in women are present. I incorporate sex-specific risk factors for stroke in the risk ascertainment component of women's medical records. With regard to depression, at minimum I perform screening for all women with coronary heart disease with a 2-item Patient Health Questionnaire (PHQ-2). For women with suspected ischemic heart disease, I adhere to the recommendations of the 2014 Consensus Statement of the AHA, "The Role of Noninvasive Testing in the Evaluation of Women with Suspected Ischemic Heart Disease." An unmet need remains an updated guideline on Prevention of Cardiovascular Disease in Women.

Cardiovascular disease prevention in women: reducing the major threat to women's health.

Cardiovascular disease prevention in women: reducing the major threat to women's health.

Myocardial Infarction and Pulmonary Arterial Hypertension in a Young Patient with Systemic Lupus Erythematosus

Systemic Lupus Erythematosus (SLE) is an autoimmune collagen vascular disease, which produces widespread damage to multiple organs. The spectrum of cardiac vascular involvement in young SLE is not elucidated completely. This report mainly describes a 27 year old girl with 20 year history of SLE presenting with both AMI (acute myocardial infarction) and PAH. Coronary angiography shows stenosis in LAD (left anterior descending artery), and occlusion in LCX (left circumflex artery). The right heart catheterization showed elevated pulmonary artery pressure (93/32/57 mmHg); acute vasoreactivity testing was negative to adenosine. Prothrombotic conditions such as homocysteine, anti-cardiolipin antibody, lupus anticoagulant levels were all normal. This is the first case that CAD and PAH happened together in one lupus patient in literature reported.

Is scleroderma a vasculopathy?

Described as an autoimmune collagen vascular disease, the most striking feature of scleroderma may be a systemic vasculopathy. This vasculopathy includes characteristic noninflammatory macrovascular and microvascular changes with dramatic and possibly occlusive formation of a thickened neointima. Scleroderma vessels also have an unusual endothelial phenotype, with loss of normal markers including vascular endothelial (VE)-cadherin. These endothelial cells express type 1 interferon and regulator of G protein signaling 5 (RGS5), two molecules associated with vascular rarefaction. These genes may be important because tissue is hypoxic with high levels of vascular endothelial growth factor (VEGF), especially early in the disease. The combination of VEGF and rarefaction is not necessarily paradoxical. VEGF-mediated angiogenesis creates labile vessels that may not survive unless the vessel acquires a smooth muscle coat. The combination of interferon and RGS5 is consistent with an antiangiogenic phenotype. We offer a hypothesis that places vascular injury at the center of this disease and also suggest possible clinical approaches for arresting and/or reversing the disease.

Other Immune Thrombocytopenias

Immune thrombocytopenic purpura (ITP) can be classified as primary (known also as idiopathic thrombocytopenic purpura) or as secondary to an underlying condition such as a malignant or nonmalignant disorder. Commonly occurring conditions associated with secondary ITP include lymphoproliferative disorders (chronic lymphocytic leukemia [CLL], Hodgkin's disease and non-Hodgkin's lymphomas), autoimmune collagen vascular diseases (systemic lupus erythematosus [SLE], thyroid disease, antiphospholipid syndrome [APS]), and chronic infections (human immunodeficiency virus [HIV], Helicobacter pylori, hepatitis C virus [HCV]). The mechanism of platelet destruction in thrombocytopenias associated with lymphoproliferative disorders and collagen vascular diseases is identical to the autoimmune mechanism seen in primary ITP. Drug-induced thrombocytopenias are uncommon and generally resolve quickly upon drug discontinuation, but are often attributed to other causes. Platelet destruction in infection-associated ITP occurs via various mechanisms including accelerated platelet clearance due to immune complex disease as seen in HIV infection or cross-reactivity of anti-platelet glycoprotein antibodies and viral antigens in HIV, HCV, and H pylori infections (antigenic mimicry). In patients with HCV-related cirrhotic liver disease, splenic sequestration secondary to portal hypertension and decreased production of thrombopoietin may further contribute to development of thrombocytopenia. The current treatment paradigm for secondary ITP varies according to the underlying condition. Standard treatments for primary ITP (corticosteroids, IVIG, anti-D, splenectomy) are often successful in secondary ITP. In cases of ITP with H pylori and HCV infection, treatment should focus on the underlying disorder.

Donor CD4+ T and B cells in transplants induce chronic graft-versus-host disease with autoimmune manifestations

AbstractChronic graft-vs-host disease (GVHD) is a major cause of morbidity and mortality of long-term survivors of allogeneic hemato-poietic cell transplantation (HCT). Chronic GVHD can have features of an autoimmune collagen vascular disease with clinical manifestations similar to autoimmune scleroderma and systemic lupus erythematosus (SLE). However, the pathogenesis of chronic GVHD is poorly understood. It is unclear how autoreactive T and B cells are generated in chronic GVHD recipients. We have recently developed a new chronic GVHD model by transplantation of donor DBA/2 (H-2d) spleen cells into major histocompatibility complex (MHC)-matched but minor antigen-mismatched sublethally irradiated BALB/c (H-2d) recipients as well as athymic BALB/cnu/nu and adult-thymectomized BALB/c recipients. Both euthymic and athymic BALB/c recipients developed high levels of serum IgG autoantibodies, sclerodermatous skin damage, and glomerulonephritis. Disease induction required both donor CD25-CD4+ T and B cells in transplants. In contrast, donor CD25+CD4+ T regulatory (Treg) cells prevented the disease induction. These results indicate that host thymus is not required for induction of chronic GVHD and that quiescent autoreactive T and B cells in transplants from nonautoimmune donors may be activated and expanded to cause chronic GVHD with autoimmune manifestations in allogeneic recipients, and donor Treg cells can suppress this process.

Donor CD4+ T and B Cells in Transplants Induce Autoimmune-Like Chronic Graft Versus Host Disease.

Chronic graft-vs-host disease (GVHD) is a major morbidity and mortality of long-term survivors of allogeneic hematopoietic cell transplantation (HCT). Chronic GVHD can have features of an autoimmune collagen-vascular disease with clinical manifestations similar to autoimmune scleroderma and systemic lupus erythematosus (SLE). However, the pathogenesis of chronic GVHD is poorly understood. It is unclear how autoreactive T and B cells are generated in chronic GVHD recipients. We have recently developed a new autoimmune-like chronic GVHD model by transplantation of donor DBA/2 (H-2d) spleen cells into MHC-matched but minor antigen-mismatched sublethally irradiated BALB/c (H-2d) recipients, as well as athymic BALB/c nu/nu and adult thymectomized BALB/c recipients. Both euthymic and athymic BALB/c recipients developed high-levels of serum IgG autoantibodies, sclerodermatous skin damage and glomerulonephritis. Disease induction required both donor CD25−CD4+ T and B cells in transplants. In contrast, donor CD25+CD4+ T regulatory (Treg) cells prevented the disease induction. These results indicate that host thymus is not required for induction of autoimmune-like chronic GVHD, and that quiescent autoreactive T and B cells in transplants from non-autoimmune donors may be activated and expanded to cause autoimmune-like chronic GVHD in allogeneic recipients, and donor Treg cells can suppress this process.

A unique case of sarcoidosis with coexistent collagen vascular disease: Possible result of a compatible disease-sustaining immunologic environment

A patient presented with dysphagia, arthralgias, and a peculiar skin eruption characterized by histopathologic features of sarcoidal granulomas and lupus erythematosus occurring in the same lesion. Sarcoidal granulomas were also found in skeletal muscle. The unique histopathologic features of this case suggest that coexistence of sarcoidosis with autoimmune collagen vascular diseases may be more than coincidence. A review of the immunologic status of patients with sarcoidosis and autoimmune collagen vascular disorders is presented with speculations on the relevance of potential disease-sustaining immunologic patterns of both groups of diseases.

Systemic Lupus Erythematosus

Systemic lupus erythematosus is a chronic systemic autoimmune collagen vascular disease that can present itself in many forms throughout any system in the body. Classification, etiology, diagnosis, course of the illness with flares and remission, prognosis, treatment and emotional coping are covered. Five tables outline lab tests, classification of symptoms by tissues primarily affected, drugs used, dietary suggestions and symptoms which need the attention of a health care provider.