An autoimmune disease is perhaps one of the cruelest of all diseases. The body's normal immune system loses its ability to recognize its own cells and attacks itself. The result is a devastating disease process which spares no organ. This group of diseases is sometimes referred to as collagen vascular diseases because the soft tissue supporting tissues and blood vessels are frequently attacked.
Anti-CCP
ANCA
Anti-Phospholipid Antibody Syndrome (Lupus Anticoagulant)
Autoimmune Progesterone Dermatitis
Churg-Strauss (Allergic Granulomatosis)
Eosinophilic Fasciitis
Dermatomyositis/Polymyositis
Goodpasture's Syndrome
Interstitial Granulomatous Dermatitis with Arthritis
Lupus Erythematosus (SLE, DLE, SCLE)
Mixed Connective Tissue Disease
Relapsing Polychondritis
Rheumatoid Arthritis
Sarcoidosis
Scleroderma
Sjogren's Syndrome
Skin Immunofluorescence
VasculitisIt should be noted that this list is not complete and indeed, there are many diseases found in other body sites which are also autoimmune in nature. A good example is pemphigus vulgaris, a devastating blistering disease of the skin and mucous membranes. Vasculitis, or inflammation of the blood vessels, is also frequently autoimmune. These latter diseases are found in the skin rash and blood vessel pages.
Anti-nuclear antibodies are directed against nuclear antigens. They are one of the hallmarks of an autoimmune disease. They can be broadly grouped into four categories.
1. Antibodies to DNA
2. Antibodies to histone
3. Antibodies to nonhistone proteins bound to RNA
4. Antibodies to nucleolar antigensThese patterns are generated by employing an enzyme-linked immunoabsorbent assay (ELISA). The immunofluorescence ANA pattern is generated by the overlay of patient serum on a Hep2 cell or mouse epithelial cell-bearing glass slide. This pattern correlates with the antibody specificity and direct immunofluorescence findings in the skin biopsy material.
Five basic patterns are recognized in indirect immunofluorescence:
Pattern Ab Directed Against Associations Homogeneous or diffuse nuclear staining Chromatin
Histones
Occasionally DS DNASLE
Drug induced SLERim or peripheral staining DS DNA SLE Fine Speckled nuclear pattern Non-DNA nuclear constituents (Extractable nuclear antigens-ENA):
Sm
Ku
U1RNP
Ro/SS-A
Ro/SS-BSCLE Coarse Speckled nuclear pattern Anti-centromere Scleroderma Nucleolar Nucleolar RNA Systemic sclerosis Associations
Antigen Antibody System Disease % Positive Native DNA DS DNA SLE 40-60% Histones Antihistone Drug induced LE >95% Core proteins of small nuclear ribonucleoprotein particles (Smith antigen) Anti-Sm SLE 20-30% Ribonucleoprotein (U1RNP) Nuclear RNP Weak SLE in 30-40% RNP SS-A (Ro)SS-B (La) Sjogren Syndrome 70-90%
Ro/SSA antigen is a 60 kDa protein attached to small RNAs to produce a 100 kDa complex with nuclear localization in normal adult skin
If insult such as UV, viral infection, or altered cytosolic calcium milieu occurs, Ro/SSA particles manifest upregulated or displaced expression from the nucleus to the cytosol and from the cytosol to the cell surface
This may explain the photosensitivity or photoreproduceability of skin lesions in anti-Ro/SSA positive LE patients and the worsening of disease symptoms in connective tissue disease patients experiencing viral illness
DNA topoisomerase I ScI-70 Systemic sclerosis 28-70% Centromeric proteins Anticentromere CREST 90% Histidyl-t-RNA synthetase Jo-1 Inflammatory myopathies 25% Conditions Where ANA is intrinsic to the Diagnostic Criteria
Drug induced SLE 100% Autoimmune hepatic disease 100% Mixed connective tissue disease 100% Diseases where ANA is useful for monitoring or prognosis
Juvenile chronic oligoarticular arthritis with uveitis 20-50% Raynaud phenomenon 20-60% Diseases where ANA is not useful in diagnosis
Rheumatoid arthritis 30-50% Multiple sclerosis 25% Idiopathic thrombocytopenia purpura 10-30% Thyroid disease 30-50% Discoid lupus erythematosus 5-25% Infections Variable Malignancies Variable Silicone breast implant patients 15-25% Fibromyalgia 15-25% Relatives of patients with autoimmune disorders 5-25% Normal Persons
Titers > than or = to Percentage 1:40 20-30 1:80 10-12 1:160 5 1:320 3 OUTLINE
Reference Methods Clinical Utility Interfering Diseases or Substances that Alter Levels Commonly Used Terms Internet Links
J Cutan Pathol 2001;28:1-23.
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
HLA-Human Leukocyte Antigen. These are a group of proteins present on the majority of cells. They interact with the body's immune system, the T lymphocytes and involved in the induction and regulation of the system. The antigens are involved in transplant rejection as well as processing and eventual elimination of foreign proteins. Many diseases, including autoimmune disorders, are associated with these disease states.
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Last Updated 1/23/2003
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