Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Chapter 59 Disorders of Musculoskeletal Function: Rheumatic Disorders

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Arthritis Primary arthritis –Affecting body systems in addition to the musculoskeletal system –Results from an immune response Secondary arthritis –Rheumatoid conditions limited to a single or few diarthrodial joints –Results from a degenerative process and the ensuing joint irregularities that occur as the bone attempts to remodel itself

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Systemic Autoimmune Rheumatic Diseases Rheumatoid arthritis Systemic lupus erythematosus Systemic sclerosis/scleroderma Polymyositis Dermatomyositis

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Characteristics of Rheumatoid Arthritis Associated with extra-articular as well as articular manifestations Usually has an insidious onset marked by systemic manifestations such as fatigue, anorexia, weight loss, and generalized aching and stiffness Characterized by exacerbations and remissions May involve only a few joints for brief durations, or it may become relentlessly progressive and debilitating

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Results of Progressive Joint Destruction May lead to subluxation (dislocation of the joint, resulting in misalignment of the bone ends) Instability of the joint Limitation of movement

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Symptoms of RA Fatigue Weakness Anorexia Weight loss Low-grade fever Anemia

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Systemic Autoimmune Rheumatic Diseases A group of chronic disorders characterized by diffuse inflammatory lesions and degenerative changes in connective tissue –These disorders share similar clinical features and may affect many of the same organs.

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins American Rheumatism Association Criteria for Rheumatoid Arthritis Morning stiffness at least 1 hour present for at least 6 weeks Swelling of three or more joints for at least 6 weeks Swelling of wrist, metacarpophalangeal, or proximal interphalangeal joints for 6 or more weeks Systemic joint swelling Hand roentgenogram changes typical of RA Rheumatoid nodules Serum rheumatoid factor

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Treatment Goals for a Person With RA Reduce pain Minimize stiffness and swelling Maintain mobility Become an informed health care consumer

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Question Is the following statement true or false? Rheumatoid arthritis is a condition of individual joint deterioration and breakdown.

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer False: RA usually has an insidious onset marked by systemic manifestations such as fatigue, anorexia, weight loss, and generalized aching and stiffness. It may involve joints sporadically or progressively.

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Characteristics of Systemic Lupus Erythematosus (SLE) Formation of autoantibodies and immune complexes B-cell hyperreactivity Increased production of antibodies against self (autoantibodies) and non-self antigens

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Characteristics of Systemic Lupus Erythematosus (SLE) (cont.) The autoantibodies can directly damage tissues or combine with corresponding antigens to form tissue-damaging immune complexes −Types of autoantibodies Antinuclear antibodies Other antibodies Platelets Coagulation factors Red blood cell surface antigens

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Categories of Clinical Manifestations of SLE Constitutional Musculoskeletal Dermatologic Cardiovascular Pulmonary Renal Neuropsychiatric

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Systemic Sclerosis Autoimmune disease of connective tissue characterized by excessive collagen deposition in the skin and internal organs Diffuse or generalized form –Skin changes involve the trunk and proximal extremities. Limited or CREST variant –Hardening of the skin (scleroderma) is limited to the hands and face.

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Manifestations of the CREST Syndrome Calcinosis (calcium deposits in the subcutaneous tissue that erupt through the skin) Raynaud phenomenon Esophageal dysmotility Sclerodactyly (localized scleroderma of the fingers) Telangiectasia

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Types of Seronegative Spondyloarthropathies Inflammation and involvement of the peripheral joints with an absence of rheumatoid factor –Ankylosing spondylitis Juvenile ankylosing spondylitis –Reactive arthritis, enteropathic arthritis (i.e., inflammatory bowel disease) –Psoriatic arthritis

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Methods of Assessing Mobility and Detecting Sacroiliitis Pressure on the sacroiliac joints with the person in a forward-bending position elicit pain and muscle spasm. Measurement of the distance between the tips of fingers and the floor in a bent-over position with straight knees Modified Schöber’s test, in which contralateral flexion of the back is measured Measurement of chest expansion may be used as an indirect indicator of thoracic involvement

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Reiter Syndrome Clinical manifestation of reactive arthritis Accompanied by extra-articular symptoms such as uveitis, bowel inflammation, carditis Develops in a genetically susceptible host after a bacterial infection due to Chlamydia trachomatis in the genitourinary tract Salmonella, Shigella, Yersinia, or Campylobacter in the gastrointestinal tract

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Subgroups of Psoriatic Arthritis Oligoarticular or asymmetric (48%) Spondyloarthropathy (24%) Polyarticular or symmetric (18%) Distal interphalangeal (8%) Mutilans (2%)

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Question Which of the following conditions is caused by collagen deposition? a.Rheumatoid arthritis b.Systemic lupus erythematosus c.Psoriatic arthritis d.Systemic sclerosis

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer a.Rheumatoid arthritis b.Systemic lupus erythematosus c.Psoriatic arthritis d.Systemic sclerosis: Systemic sclerosis is an autoimmune disease of connective tissue characterized by excessive collagen deposition in the skin and internal organs.

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Osteoarthritis Degenerative joint disease Primary variants of OA occur as localized or generalized syndromes Secondary OA has a known underlying cause such –Congenital or acquired defects of joint structures, trauma, metabolic disorders, or inflammatory diseases

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Causes of Osteoarthritis Postinflammatory diseases Post-traumatic disorders Anatomic or bony disorders Metabolic disorders Neuropathic arthritis Hereditary disorders of collagen Idiopathic or primary variants

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Osteoarthritis-Induced Joint Changes Progression –A progressive loss of articular cartilage –Synovitis –Osteophytes Bone spurs Manifestations –Joint pain –Stiffness –Limitation of motion –Joint instability –Deformity

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Metabolic and Endocrine Diseases Associated With Joint Symptoms Amyloidosis Gout syndrome Osteogenesis imperfecta Diabetes mellitus Hyperparathyroidism Thyroid disease AIDS Hypermobility syndromes

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Gout Syndrome Acute gouty arthritis with recurrent attacks of severe articular and periarticular inflammation Tophi or the accumulation of crystalline deposits in articular surfaces, bones, soft tissue, and cartilage Gouty nephropathy or renal impairment Uric acid kidney stones

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Types of Gout Primary gout –Designate cases in which the cause of the disorder is unknown or an inborn error in metabolism –Characterized primarily by hyperuricemia and gout Secondary gout –The cause of the hyperuricemia is known, but the gout is not the main disorder

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Objectives for Treatment of Gout Termination and prevention of the acute attacks of gouty arthritis Correction of hyperuricemia Inhibition of further precipitation of sodium urate Absorption of urate crystal deposits already in the tissues

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Juvenile Idiopathic Arthritis (JIA) JIA categories of diseases with three principal types of onset: 1.Systemic onset disease 2.Pauciarticular arthritis 3.Polyarticular disease Systemic symptoms –Low-grade fever –Stunted growth –Weight loss –Malaise

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Juvenile Rheumatoid Arthritis (JRA) Definition –A chronic disease characterized by synovitis Manifestations –Can influence epiphyseal growth by stimulating growth of the affected side –Generalized stunted growth also may occur

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Manifestations of Children With SLE Constitutional symptoms –Fever, malaise, anorexia, weight loss Symptoms of the integumentary, musculoskeletal, central nervous, cardiac, pulmonary, and hematopoietic systems are similar to those of adults. Endocrine abnormalities include –Cushing syndrome from long-term corticosteroid use –Autoimmune thyroiditis

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Signs and Symptoms of Giant Cell Arteritis Constitutional symptoms –Malaise, fatigue, fever, weight loss, cough, sore throat Polymyalgia rheumatica syndrome Manifestations related to vascular involvement Ischemic optic neuropathy Claudication of jaw or arm

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Question Uric acid accumulation is involved in which condition? a.Amyloidosis b.Gout syndrome c.Osteogenesis imperfecta d.Diabetes mellitus e.Hyperparathyroidism

Copyright © 2009 Wolters Kluwer Health | Lippincott Williams & Wilkins Answer a.Amyloidosis b.Gout syndrome: Gout is the result of hyperuricemia. c.Osteogenesis imperfecta d.Diabetes mellitus e.Hyperparathyroidism