The Major Histocompatibility Complex (The Immune System in Health and Disease)(Rheumatology) Part 3

Biosynthesis and Function of Class II Molecules The intracellular assembly of class II molecules occurs within a specialized compartmentalized pathway that differs dramatically from the class I pathway described above. As illustrated in Fig. 2-3B, the class II molecule assembles in the ER in association with a chaperone molecule, known as the invariant chain. The […]

Autoimmunity and Autoimmune Diseases (The Immune System in Health and Disease) (Rheumatology) Part 1

One of the classically accepted features of the immune system is the capacity to distinguish self from nonself. Although animals are able to recognize and generate reactions to a vast array of foreign materials, most do not mount immune responses to self-antigens under ordinary circumstances and thus are tolerant to self. Whereas recognition of self […]

Autoimmunity and Autoimmune Diseases (The Immune System in Health and Disease) (Rheumatology) Part 2

Autoimmune Diseases Manifestations of autoimmunity are found in a large number of pathologic conditions. However, their presence does not necessarily imply that the pathologic process is an autoimmune disease. A number of attempts to establish formal criteria for the diagnosis of autoimmune diseases have been made, but none is universally accepted. One set of criteria […]

Systemic Lupus Erythematosus (Disorders of Immune-Mediated Injury) (Rheumatology) Part 1

Definition and Prevalence Systemic lupus erythematosus (SLE) is an autoimmune disease in which organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes. Ninety percent of patients are women of child-bearing years; people of both sexes, all ages, and all ethnic groups are susceptible. Prevalence of SLE in the United States is 15-50 […]

Systemic Lupus Erythematosus (Disorders of Immune-Mediated Injury) (Rheumatology) Part 2

Diagnosis The diagnosis of SLE is based on characteristic clinical features and autoantibodies. Current criteria (2006) for classification are listed in Table 4-3 (new criteria are being developed), and an algorithm for diagnosis and initial therapy is shown in Fig. 4-2. The criteria are intended for confirming the diagnosis of SLE in patients included in […]

Systemic Lupus Erythematosus (Disorders of Immune-Mediated Injury) (Rheumatology) Part 3

Nervous System Manifestations There are many central nervous system (CNS) and peripheral nervous system manifestations of SLE; in some patients these are the major cause of morbidity and mortality. It is useful to approach this diagnostically by asking first whether the symptoms result from SLE or another condition (such as infection in immunosuppressed individuals). If […]

Systemic Lupus Erythematosus (Disorders of Immune-Mediated Injury) (Rheumatology) Part 4

Life-Threatening SLE:  Proliferative FORMS OF LUPUS NEPHRITIS The mainstay of treatment for any inflammatory life-threatening or organ-threatening manifestations of SLE is systemic glucocorticoids (0.5-2 mg/kg per day PO or 1000 mg of methylprednisolone sodium succinate IV daily for 3 days followed by 0.5-1 mg/kg of daily prednisone or equivalent). Evidence that glucocorticoid therapy is life-saving […]

Rheumatoid Arthritis (Disorders of Immune-Mediated Injury) (Rheumatology) Part 1

Rheumatoid arthritis (RA) is a chronic multisystem disease of unknown cause. Although there are a variety of systemic manifestations, the characteristic feature of established RA is persistent inflammatory synovitis, usually involving peripheral joints in a symmetric distribution. The potential of the synovial inflammation to cause cartilage damage and bone erosions and subsequent changes in joint […]

Rheumatoid Arthritis (Disorders of Immune-Mediated Injury) (Rheumatology) Part 2

Clinical Manifestations Onset Characteristically, RA is a chronic polyarthritis. In approximately two-thirds of patients, it begins insidiously with fatigue, anorexia, generalized weakness, and vague musculoskeletal symptoms until the appearance of synovitis becomes apparent. This prodrome may persist for weeks or months and defy diagnosis. Specific symptoms usually appear gradually as several joints, especially those of […]

Rheumatoid Arthritis (Disorders of Immune-Mediated Injury) (Rheumatology) Part 3

Clinical Course and Prognosis The course of RA is quite variable and difficult to predict in an individual patient. The current therapeutic approach of early aggressive intervention appears to have mitigated the clinical course of RA, resulting in less persistent inflammation, disability, joint damage, and mortality. Classically, most patients had experienced persistent but fluctuating disease […]