Primer on the Rheumatic Diseases Download book PDF. Chapters Table of PDF · Molecular and Cellular Basis of Immunity and Immunological Diseases. Primer on the Rheumatic Diseases DRM-free; Included format: PDF; ebooks can be used on all reading devices; Immediate eBook download after download. We Can Help!" Primer on the Rheumatic Diseases THIRTEENTH EDITION Primer on the Rheumatic Diseases THIRTEENTH EDITION Edited by John H. Klippel.
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INTRODUCTIONThis is the fourth edition of the Primer. The first was published by the American Committee for the Control of Rheumatism in in cooperation. A note on the genealogy of the Primer: This is the sixth Primer on the Rheumatic Diseases to be published in The Journalof the American Medical. copies Request PDF on ResearchGate | Primer on the rheumatic diseases: Thirteenth edition | Arthritis and other rheumatic diseases are a leading.
Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Albrecht K, Zink A. Poor prognostic factors guiding treatment decisions in rheumatoid arthritis patients: a review of data from randomized clinical trials and cohort studies.
Meta-analysis: diagnostic accuracy of anti-cyclic citrullinated peptide antibody and rheumatoid factor for rheumatoid arthritis. Ann Intern Med. Shojania K. Rheumatology: 2.
What laboratory tests are needed?. The rheumatoid factor: an analysis of clinical utility. Am J Med. Lack of seroconversion of rheumatoid factor and anti-cyclic citrullinated peptide in patients with early inflammatory arthritis: a systematic literature review. Rheumatology Oxford. How to diagnose rheumatoid arthritis early: a prediction model for persistent erosive arthritis.
The role of antineutrophil cytoplasmic antibody c-ANCA testing in the diagnosis of Wegener granulomatosis. A literature review and meta-analysis. Vassilopoulos D, Hoffman GS. Clinical utility of testing for antineutrophil cytoplasmic antibodies. Clin Diagn Lab Immunol. Role of human leukocyte antigens HLA in autoimmune disease [published online ahead of print March 7, ]. Rheumatol Ther. Accessed April 16, The erythrocyte sedimentation rate.
Guidelines for rational use. Ralph, MD et al.
Rheumatoid Arthritis Tehlirian, Christopher V. Psoriatic Arthritis Gladman, Dafna D. Gout Edwards, N. Lawrence, MD Pages Systemic Sclerosis Mayes, Maureen D. Metabolic Myopathies Baer, Alan N. Vasculitides Weyand, Cornelia M. Relapsing Polychondritis Luthra, Harvinder S. Periodic Syndromes Ryan, John G.
Sarcoidosis Chen, Edward S.
Neoplasms of the Joint Cooper, Andrew J. Osteonecrosis Seyler, Thorsten M. Osteoporosis Saag, Kenneth G. Pain Management Winfield, John B.
A serum ANA is present in some healthy people, especially the elderly. Because of its high sensitivity, the ANA is most useful when it is negative. A negative test result nearly excludes the diagnosis of SLE. This persistent elevated ANA titer should not be used as an indicator for initiating therapy. When the ANA test is positive, the fluorescent pattern may be somewhat helpful in subclassifying the rheumatic diseases.
Four types of patterns of nuclear fluorescence have been described in which the titer is also provided. No single pattern has been found to be diagnostic of any specific disease, but there have been strong associations.
Additionally, all or any combination of patterns may appear simultaneously, and any one pattern may somewhat obscure the others. The four patterns of ANA fluorescent nuclear staining are homogeneous diffuse , speckled, peripheral rim , and nucleolar. See Table 2. The nucleolar pattern is less frequently seen but is more commonly associated with scleroderma and rheumatologic overlap syndromes with sclerodermatous features. If the clinician suspects a diagnosis of SLE or a disease process with clinical features of SLE in which the ANA result is positive, then further antibody tests are warranted.
The anti-dsDNA antibody test does not predict lupus nephritis nor other particular SLE disease manifestations, but because these antibodies often fluctuate in parallel with disease activity, test results are used as an SLE disease monitor. Anti-Smith Antibody. The anti-Sm antibody test does not correlate with any particular feature of SLE nor does it vary greatly with time.
The anti-smooth muscle antibody test is associated with autoimmune chronic active hepatitis and other liver diseases. Therefore, writing out "anti-Smith" instead of "anti-Sm" may ensure that the correct test is performed.
Anti-Ribonucleoprotein Antibody. In , Sharp et al described a group of patients with overlapping clinical features of SLE, polymyositis, and scleroderma and coined the term "mixed connective tissue disease" or MCTD. Patients also have high serum titers of speckled ANA. Antibodies to ribosomal RNP most often account for cytoplasmic staining in immunofluorescence.
These antibodies are called "extractable nuclear antigens" or ENAs, because they are easily extractable in normal saline solution. There are more than 20 different anti-ENA antibodies described, but the two major ones are anti-U1snRNP, and the anti-Sm, which has been previously discussed above.
High titers do not seem to be associated with worse disease. However, in patients with these overlapping features, if the serum is negative for anti-RNP antibodies, the term "undifferentiated connective tissue disease" is used for the diagnosis. A laboratory should have a listing of the component tests offered on each panel.
However, rather than ordering a panel, it is better to order the necessary tests individually. Anti-ribosomal P Antibody. The clinician considering ordering the anti-ribosomal P antibody test should remember that the results obtained may not help to support or rule out CNS lupus and consequently will not alter the diagnosis nor the planned course of treatment.
Children born to mothers with a positive serum anti-SSA antibody test run a significant risk of having neonatal heart block, which will be discussed below under "Neonatal Lupus. This subset of SLE patients with a photosensitive, non-scarring skin rash with either annular or psoriasiform morphology tends to have less frequent visceral involvement. Neonatal Lupus. Neonatal lupus syndrome present at birth, or shortly thereafter, is characterized by a lupus skin rash, transient or permanent congenital heart block, cytopenias, and liver inflammation.
The mother of the infant has SLE or some form of systemic connective tissue disease and tests positive for the anti-SSA antibody.
Because of transplacental transfer of maternal IgG, the infant tests positive for anti-SSA antibodies. The risk of having a child with the syndrome is higher if the mother has both antibodies to SSA and SSB, but the risk is lower if the mother has isolated SSA in low titers.
Careful monitoring of the fetus should be done before as well as after delivery. A negative anti-histone antibody test is used to help rule out a clinical diagnosis of drug-induced lupus. The clinical features of drug-induced lupus are often less severe than those of SLE, of which the most commonly reported symptoms are fever, arthritis, and serositis. The complement system is a group of proteins that functions as a mediator of inflammation. The protein components interact with each other and with other aspects of the immune system.
Complement activation is a cascade reaction.
Patients with active SLE, especially with nephritis, frequently show evidence in their serum of activitation of the complement pathway by depression of the levels of C3, C4, and CH Lowered serum complement levels may indicate both consumption of components and a decrease in complement synthesis as in lupus nephritis.
It is not practical and cost-effective to measure most of the complement components because they are unstable. CH50 and C3 are the most useful initial measures of the complement system.
The CH50 is a measure of the integrity of the entire classical complement cascade, but not the alternative pathway. C3 is the most stable complement component, and its depression indicates activity in either or both classical and alternative pathways. C4 is also a measure of the classic complement pathway and may also be low in active SLE.
However, C4 by itself may not be an accurate indication of SLE activity, as it is commonly low in people who have an inherited deficiency of C4. The prevalence of complement deficiency in the general population is estimated at 0.
See Table 4. Cardiolipin is a phospholipid that was isolated in from beef heart by Mary Pangborn during investigations of serologic tests for syphilis. These individuals usually do not develop SLE. Antiphospholipid APL antibodies can either be drug induced or autoimmune. A positive LAC test is suggested by the following clinical findings: 1 A prolonged partial thromboplastin time PTT ; 2 failure to correct the abnormal clotting time by mixing patient serum with normal serum suggesting the presence of a clotting inhibitor ; and 3 normalization of the test with freeze-thawed platelets or phospholipids.
The major features of WG include a triad of necrotizing granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis.
To confirm the diagnosis in patients with suspected WG and rule out other processes, such as systemic infections and malignancies, it is essential to have tissue evidence, preferably from an open lung biopsy.
C-ANCA is identified by a coarse, granular, centrally-accentuated pattern that decreases in intensity toward the periphery of the cell. Proteinase 3 is a kd serine protease found in the azurophilic granules of the neutrophil and is the major antigen for C-ANCA. Ethanol causes the rearrangement of positively charged granule constituents around and on the negatively charged nuclear membrane, resulting in an artifactual perinuclear pattern.
Scleroderma Antibodies Scleroderma or systemic sclerosis SSc is a connective tissue disease in which there is fibrosis of the skin and visceral organs. The disease is rare in childhood and peaks at years of age. There is a preponderance for females, although the female-to-male ratio varies with age, the highest being during childbearing years.
Isolated cases of SSc may be secondary to exposure to vinyl-chloride, benzene, or the chemotherapeutic agent, bleomycin. Diffuse scleroderma includes the distal and proximal extremities, the internal organs, the face, and the trunk of the body. The laboratory hallmarks of these muscle diseases are elevated serum creatine kinase CK and aldolase levels.
The LDH and transaminases may also be elevated. There are reported cases of patients with normal CK and serum aldolase levels but with muscle biopsies positive for the presence of an inflammatory myopathy. An electromyelogram EMG is a sensitive test for evaluating an inflammatory myopathy. However, there is little correlation between the amount of weakness or functional disability and the EMG findings.
See Table 6 for a brief differential diagnosis of muscle weakness. Patients present with characteristic symptoms of dry eyes xerophthalmia , dry mouth xerostomia , and, often, parotid gland swelling and vaginal dryness.
A minor salivary gland biopsy may also be useful. In the s, Waaler and Rose and colleagues discovered that the majority of sera of patients with rheumatoid arthritis RA agglutinated sheep red blood cells sensitized with rabbit anti-erythrocyte antibodies. RFs are autoantibodies directed against antigenic determinants on the Fc fragment of IgG.
IgM RFs are multivalent and are efficient agglutinators of antigen-coated particles. RF tests can be performed by nephelometry or agglutination. There is some variation in the sensitivity of RF detection depending on which technique is used.
Agglutination using latex particles or bentonite is more sensitive but less specific than tests that employ sheep red cells. The pathologic role of RF in RA remains unclear. The RF titer does not correlate with disease activity, but it is generally considered that high titer RFs are associated with more severe disease, including the presence of rheumatoid nodules and extra-articular features. The RF test sub-classifies RA patients into seropositive and seronegative disease.
Patients with "RA" but negative for RF need to be watched more closely for the development of a different diagnosis, such as psoriatic arthritis or one of the spondyloarthropathies.