Pathogenesis of macrophage activation syndrome and potential for cytokine- directed therapies.

Abstract:

:Macrophage activation syndrome (MAS) is an acute episode of overwhelming inflammation characterized by activation and expansion of T lymphocytes and hemophagocytic macrophages. In rheumatology, it occurs most frequently in patients with systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus. The main clinical manifestations include cytopenias, liver dysfunction, coagulopathy resembling disseminated intravascular coagulation, and extreme hyperferritinemia. Clinically and pathologically, MAS bears strong similarity to hemophagocytic lymphohistiocytosis (HLH), and some authors prefer the term secondary HLH to describe it. Central to its pathogenesis is a cytokine storm, with markedly increased levels of numerous proinflammatory cytokines including IL-1, IL-6, IL-18, TNFα, and IFNγ. Although there is evidence that IFNγ may play a central role in the pathogenesis of MAS, the role of other cytokines is still not clear. There are several reports of SJIA-associated MAS dramatically benefiting from anakinra, a recombinant IL-1 receptor antagonist, but the utility of other biologics in MAS is not clear. The mainstay of treatment remains corticosteroids; other medications, including cyclosporine, are used in patients who fail to respond.

journal_name

Annu Rev Med

authors

Schulert GS,Grom AA

doi

10.1146/annurev-med-061813-012806

subject

Has Abstract

pub_date

2015-01-01 00:00:00

pages

145-59

eissn

0066-4219

issn

1545-326X

journal_volume

66

pub_type

杂志文章,评审
  • Immune disregulation of hematopoiesis.

    abstract::In vitro coculture studies of normal T lymphocytes, natural killer (NK) cells, or monocytes with human hematopoietic progenitor cells (HPC) suggest a potential role for immune cells in the regulation of hematopoiesis. In some patients with severe aplastic anemia or selective pure red cell, white cell, or megakaryocyte...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.me.38.020187.000425

    authors: Mangan KF

    更新日期:1987-01-01 00:00:00

  • Leukocyte adhesion deficiency: an inherited defect in the Mac-1, LFA-1, and p150,95 glycoproteins.

    abstract::Leukocyte adhesion deficiency (LAD) is a recently recognized autosomal-recessive trait characterized by recurrent bacterial infections, impaired pus formation and wound healing, and abnormalities in a wide spectrum of adherence-dependent functions of granulocytes, monocytes, and lymphoid cells. Features of this diseas...

    journal_title:Annual review of medicine

    pub_type: 杂志文章

    doi:10.1146/annurev.me.38.020187.001135

    authors: Anderson DC,Springer TA

    更新日期:1987-01-01 00:00:00

  • The course and treatment of lupus nephritis.

    abstract::Renal involvement by systemic lupus is variable; some patients have minimal clinical and histologic involvement, whereas others have fulminant renal failure and severe proliferative renal lesions on biopsy. The World Health Organization (WHO) classification has greatly aided in the study of lupus nephritis. This class...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.45.1.525

    authors: Appel GB,Valeri A

    更新日期:1994-01-01 00:00:00

  • Recent advancements in the treatment of chronic myelogenous leukemia.

    abstract::Chronic myelogenous leukemia (CML) is a clonal hematopoetic stem cell disorder characterized by the Philadelphia chromosome and resultant production of the constitutively activated Bcr-Abl tyrosine kinase. Characterized clinically by marked myeloid proliferation, it invariably terminates in an acute leukemia. Conventi...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.53.082901.103853

    authors: O'Dwyer ME,Mauro MJ,Druker BJ

    更新日期:2002-01-01 00:00:00

  • Pathophysiology of atrial flutter.

    abstract::Atrial flutter is a macroreentrant tachyarrhythmia most often contained within the right atrium. Typical atrial flutter is defined on an electrocardiogram by the classic "sawtooth" pattern of flutter waves with negative polarity in leads II, III, and aVF. In contrast to atrial fibrillation, which is sustained by multi...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.49.1.77

    authors: Daoud EG,Morady F

    更新日期:1998-01-01 00:00:00

  • Circadian rhythms: basic neurobiology and clinical applications.

    abstract::Circadian rhythms are major features of adaptation to our environment. In mammals, circadian rhythms are generated and regulated by a circadian timing system. This system consists of entertainment pathways, pacemakers, and pace-maker output to effector systems that are under circadian control. The primary entertainmen...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.48.1.253

    authors: Moore RY

    更新日期:1997-01-01 00:00:00

  • Management of adult idiopathic thrombocytopenic purpura.

    abstract::Idiopathic thrombocytopenic purpura (ITP) is a common hematologic disorder manifested by immune-mediated thrombocytopenia. The diagnosis remains one of exclusion, after other thrombocytopenic disorders are ruled out based on history, physical examination, and laboratory evaluation. The goal of treatment is to raise th...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.56.082103.104644

    authors: Cines DB,McMillan R

    更新日期:2005-01-01 00:00:00

  • Parenteral and enteral nutrition.

    abstract::Stress and starvation, especially when complicated by sepsis, will give rise to a rapid erosion of the cellular mass, which significantly affects morbidity and mortality. The best clinical evaluation of the nutritional state is obtained from the medical history and the physical examination. In the patient who cannot e...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.me.42.020191.003001

    authors: Shizgal HM

    更新日期:1991-01-01 00:00:00

  • Concepts of clot lysis.

    abstract::Physiologic thrombolysis is efficient, while pathologic aberrations in the fibrinolytic system may result in either thrombotic or hemorrhagic disease. This review considers the molecular interactions involved in fibrinolysis, discusses the normal control mechanisms that provide for localized activation without systemi...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.me.37.020186.001155

    authors: Francis CW,Marder VJ

    更新日期:1986-01-01 00:00:00

  • Collagenous colitis and lymphocytic colitis.

    abstract::Collagenous and lymphocytic colitis are newly recognized causes of chronic watery diarrhea that typically affect middle-aged patients. Although endoscopic studies are normal, inflammatory changes and (in the case of collagenous colitis) collagen deposition occur histologically in the colonic mucosa. The pathogenesis o...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.45.1.105

    authors: Zeroogian JM,Chopra S

    更新日期:1994-01-01 00:00:00

  • Neuromodulation techniques for medically refractory chronic pain.

    abstract::Advances in our knowledge of the physiology of pain transmission and modulation have created new surgical options for the control of chronic pain. The pain modulation network can be activated by administration of spinal opiates or by electrical stimulation of the nervous system with transcutaneous, peripheral nerve, s...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.me.44.020193.001431

    authors: Landau B,Levy RM

    更新日期:1993-01-01 00:00:00

  • Pulmonary complications of general surgery.

    abstract::1. Patients should be divided preoperatively into low- or high-risk categories, depending on their probability of developing postoperative pulmonary complications. The evaluation should include spirometry as well as an assessment of the previously defined risk factors. 2. Patients in a low-risk category need only inst...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.me.28.020177.001235

    authors: Pierce AK,Robertson J

    更新日期:1977-01-01 00:00:00

  • Chemokine antagonists as therapeutics: focus on HIV-1.

    abstract::Human immunodeficiency virus type 1 (HIV-1) entry into target cells is a multistep process involving the interaction of viral envelope proteins with cell surface receptors. Binding to CD4 is followed by engagement of specific chemokine receptors (CCR5 or CXCR4), triggering molecular rearrangements in the envelope tran...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.58.080105.102908

    authors: Tsibris AM,Kuritzkes DR

    更新日期:2007-01-01 00:00:00

  • Central nervous system effects of human immunodeficiency virus type 1.

    abstract::Following its early entry into the central nervous system (CNS), HIV-1 alters cerebral cell architecture and may subsequently affect higher cognitive functions, leading eventually in some patients to HIV-1 encephalopathy. The CNS may also be the target of opportunistic infections and malignancy secondary to HIV-1 immu...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.me.41.020190.001111

    authors: Bridge TP,Ingraham LJ

    更新日期:1990-01-01 00:00:00

  • Therapeutic approaches to hemoglobin switching in treatment of hemoglobinopathies.

    abstract::The past decade has witnessed profound increases in knowledge of the structure, function, and developmental regulation of the human globin genes. This information has deepened our understanding of the molecular and cellular mechanisms underlying inherited disorders affecting hemoglobin, and it has provided a new persp...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.me.43.020192.002433

    authors: Stamatoyannopoulos JA,Nienhuis AW

    更新日期:1992-01-01 00:00:00

  • Computed Tomography Scanning for Early Detection of Lung Cancer.

    abstract::Parallel and often unrelated developments in health care and technology have all been necessary to bring about early detection of lung cancer and the opportunity to decrease mortality from lung cancer through early detection of the disease by computed tomography. Lung cancer screening programs provide education for pa...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev-med-020917-053556

    authors: Jacobson FL,Jaklitsch MT

    更新日期:2018-01-29 00:00:00

  • Circulating tumor cells and circulating tumor DNA.

    abstract::Solid tumors derived from epithelial tissues (carcinomas) are responsible for 90% of all new cancers in Europe, and the main four tumor entities are breast, prostate, lung, and colon cancer. Present tumor staging is mainly based on local tumor extension, metastatic lymph node involvement, and evidence of overt distant...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev-med-062310-094219

    authors: Alix-Panabières C,Schwarzenbach H,Pantel K

    更新日期:2012-01-01 00:00:00

  • Ductal carcinoma in situ of the breast.

    abstract::Ductal carcinoma in situ of the breast is a heterogeneous group of lesions with diverse malignant potential. It is the most rapidly growing subgroup in the breast cancer family; it is projected that more than 39,000 new cases will be diagnosed in the United States during 1999. Most new cases are nonpalpable and are di...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.51.1.17

    authors: Silverstein MJ

    更新日期:2000-01-01 00:00:00

  • Emotion recollected in tranquility: lessons learned from the COX-2 saga.

    abstract::Nonsteroidal antinflammatory drugs (NSAIDs) inhibit prostaglandin formation by cyclooxygenases (COX) 1 and 2. NSAIDs selective for inhibition of COX-2 are less likely than traditional drugs to cause serious gastrointestinal adverse effects, but predispose to adverse cardiovascular events, such as heart failure, myocar...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev-med-011209-153129

    authors: Grosser T,Yu Y,Fitzgerald GA

    更新日期:2010-01-01 00:00:00

  • The genetics of multiple endocrine neoplasia syndromes.

    abstract::The familial nature of multiple endocrine neoplasia syndromes (FMEN) has been recognized for some time but little is known about their cause. Recent application of new molecular techniques has mapped FMEN syndromes to specific chromosomes and provided evidence for the mechanism through which neoplasia occurs. In this ...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.me.43.020192.001345

    authors: Nanes MS,Catherwood BD

    更新日期:1992-01-01 00:00:00

  • Catastrophic Antiphospholipid Syndrome: Candidate Therapies for a Potentially Lethal Disease.

    abstract::Catastrophic antiphospholipid syndrome (CAPS) is a potentially lethal disease that presents with rapidly progressive multiple organ thromboses. Anticoagulation, corticosteroids, intravenous immunoglobulin, and plasma exchange are the most commonly used treatments for CAPS patients. However, the high mortality despite ...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev-med-042915-102529

    authors: Unlu O,Erkan D

    更新日期:2017-01-14 00:00:00

  • Prospects for life span extension.

    abstract::Life expectancy has increased dramatically in the United States and in much of the world in recent years and decades. The factors underlying this increase are incompletely understood and are undoubtedly complex. A question that drives current research is whether life expectancy can be further extended using current kn...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.60.061607.220533

    authors: Sierra F,Hadley E,Suzman R,Hodes R

    更新日期:2009-01-01 00:00:00

  • Post-transplant lymphoproliferative disorders.

    abstract::Post-transplant lymphoproliferative disorder (PTLD) is a life-threatening complication after hematopoietic stem cell or solid organ transplantation. The majority of PTLD is of B-cell origin and associated with Epstein-Barr virus (EBV). During the past decade progress has been made in better understanding the pathogene...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.56.082103.104727

    authors: Gottschalk S,Rooney CM,Heslop HE

    更新日期:2005-01-01 00:00:00

  • Barrett's Esophagus and esophageal adenocarcinoma.

    abstract::The incidence of esophageal adenocarcinoma (EAC) has risen dramatically over the past three decades in western countries. The importance of Barrett's esophagus (BE) derives from its potential to transform to adenocarcinoma. BE is characterized by endoscopically recognized displacement of the squamocolumnar junction pr...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.59.061206.112706

    authors: Bresalier RS

    更新日期:2009-01-01 00:00:00

  • Alzheimer's disease.

    abstract::Significant progress has been made in identifying changes in cholinergic, monoaminergic, and peptidergic neurotransmitter systems in the brains of individuals with Alzheimer's disease. Moreover, neurobiological approaches are beginning to reveal the relationships between these neurotransmitter abnormalities and histol...

    journal_title:Annual review of medicine

    pub_type: 杂志文章

    doi:10.1146/annurev.me.36.020185.002025

    authors: Price DL,Whitehouse PJ,Struble RG

    更新日期:1985-01-01 00:00:00

  • Paget's disease of bone.

    abstract::Paget's disease affects approximately 3% of the population over age forty. It may be genetically transmitted (autosomal-dominant), but recent evidence suggests a viral etiology. Effective agents for control of the disorder have been developed over the past decade, but all have significant drawbacks. Calcitonin tends t...

    journal_title:Annual review of medicine

    pub_type: 杂志文章

    doi:10.1146/annurev.me.28.020177.001043

    authors: Ryan WG

    更新日期:1977-01-01 00:00:00

  • The changing face of tuberculosis.

    abstract::Tuberculosis (TB) remains an important public health problem worldwide, resulting in a estimated 8 to 10 million new cases and 2 to 3 million deaths each year. Between 1953 and 1985, the number of TB cases in the US declined by an average of 6% per year. However, since 1985, TB has been increasing in the US. Approxima...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.46.1.47

    authors: Huebner RE,Castro KG

    更新日期:1995-01-01 00:00:00

  • Recent advances in surgery for emphysema.

    abstract::Volume reduction surgery is based on the removal of volume-occupying but nonfunctioning emphysematous lung, which is thought to improve pulmonary elastic recoil. The reduction in thoracic volume may also improve thoracic cage and inspiratory muscle function. In addition, dyspnea is lessened, exercise tolerance is incr...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.48.1.387

    authors: Lefrak SS,Yusen RD,Trulock EP,Pohl MS,Patterson A,Cooper JD

    更新日期:1997-01-01 00:00:00

  • New approaches to the treatment of osteoporosis.

    abstract::Although safe and effective agents are currently available to treat osteoporosis, fragility fractures remain a significant problem worldwide. Recent improvements in the understanding of the cellular, biochemical, and molecular pathways of bone biology have led to the development of newer agents to treat osteoporosis, ...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev-med-061709-145401

    authors: Silva BC,Bilezikian JP

    更新日期:2011-01-01 00:00:00

  • Allergic bronchopulmonary aspergillosis.

    abstract::Allergic bronchopulmonary aspergillosis (ABPA) is a syndrome seen in patients with asthma and cystic fibrosis. It is characterized by chronic colonization of the airways with a ubiquitous fungus, Aspergillus fumigatus. The clinical expression of ABPA results from the complex interaction of chronic colonization of the ...

    journal_title:Annual review of medicine

    pub_type: 杂志文章,评审

    doi:10.1146/annurev.med.50.1.303

    authors: Cockrill BA,Hales CA

    更新日期:1999-01-01 00:00:00