Color atlas of pathology : pathologic principles, associated diseases, sequela

Contents 1 Fundamentals of Pathology Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 Methods Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Etiology Causal Pathogenesis Formal Pathogenesis Clinical Course Signs of Death . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Equivocal Signs of Death Brain Death Unequivocal Signs of Death Statistics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Autopsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 2 Cellular Pathology Nuclear Lesions . . . . . . . . . . . . . . . . . . . . . . . . 6 Forms of Nuclei Chromosomes DNA Repair Defects . . . . . . . . . . . . . . . . . . . . . 6 Xeroderma pigmentosum Chromosome Abnormalities . . . . . . . . . . . . . . 6 Change in the Size of the Nucleus . . . . . . . . 8 Relation of Nucleus to Cytoplasm Nuclear Euploidy Nuclear Polyploidy Nuclear Aneuploidy Chromatin Change . . . . . . . . . . . . . . . . . . . . . . 8 Heterochromatin Condensation Dyskaryosis Perinuclear Hyperchromatosis Karyolysis Inclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 Cytoplasmic inclusions Paraplasmic inclusions Change in the Number of Nuclei . . . . . . . . . 10 Polynuclear Cells Anuclear Cells Antinuclear Autoantibodies . . . . . . . . . . . . . 10 Nucleolar Change . . . . . . . . . . . . . . . . . . . . . . 10 Inflammatory nucleoli Tumor nucleoli Giant nucleoli Lesions of the Rough Endoplasmic Reticulum (RER) . . . . . . . . . . . . . . . . . . . . . . 12 Quantitative Change . . . . . . . . . . . . . . . . . . . 12 Increase in the number of cisterns Decrease in the number of cisterns Morphologic Change . . . . . . . . . . . . . . . . . . . 12 Fragmentation of the RER Formation of vacuoles in the RER Swelling of the RER RER cistern collapse Inclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 Amorphous inclusions Crystalline inclusions Particulate inclusions Oncofetal Lesion . . . . . . . . . . . . . . . . . . . . . . . 12 Ribosome-lamellae complexes Annulated lamellae layers Mitochondria-lamellar-layer complexes Lesions of the Smooth Endoplasmic Reticulum (SER) . . . . . . . . . . . . . . . . . . . . . . 14 Quantitative Change . . . . . . . . . . . . . . . . . . . 14 Proliferation of SER Atrophy of the SER Morphologic Change . . . . . . . . . . . . . . . . . . . 14 Formation of Vesicles in the SER „Cytoplasmic Nuclei“ Inclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14 Golgi Lesions . . . . . . . . . . . . . . . . . . . . . . . . . 16 Atrophy of the Golgi Apparatus . . . . . . . . . . 16 Hypertrophy of the Golgi Apparatus . . . . . 16 Golgi-associated disorders . . . . . . . . . . . . . . . 16 Cholestasis Fatty Liver Achondroplasia Alveolar Proteinosis Mitochondrial Lesions . . . . . . . . . . . . . . . . 18 Quantitative Change . . . . . . . . . . . . . . . . . . . 18 Mitochondrial Proliferation Mitochondrial Depletion Morphologic Change . . . . . . . . . . . . . . . . . . . 18 Megamitochondria Mitochondrial Swelling Structural Change in Mitochondrial DNA . . 20 Riede / Werner, Color Atlas of Pathology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. XII Contents Mitochondrial DNA Mutations . . . . . . . . . . . 20 Congenital Forms Acquired Forms Changes in the Mitochondrial Cristae . . . . 20 Membrane proliferation Membrane loss Inclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20 Matrix inclusions Crista inclusions Grana inclusions Mitochondrial Disorders . . . . . . . . . . . . . . 22 Mitochondrial Myopathies . . . . . . . . . . . . . . 22 Hypoxia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22 Hypoxemic Hypoxia Ischemic Hypoxia Hyperglycemic Hypoxia Histotoxic Hypoxia Mitochondrial Autoantibodies . . . . . . . . . . . 22 Peroxisomal Lesions . . . . . . . . . . . . . . . . . . 24 Peroxisomal Diseases . . . . . . . . . . . . . . . . . . . 24 Acatalasemia Refsum Disease Cerebrohepatorenal Syndrome Adrenoleukodystrophy Systemic Carnitine Deficiency Lysosomal Lesions . . . . . . . . . . . . . . . . . . . . 26 Prelysosomal Phase . . . . . . . . . . . . . . . . . . . . 26 Heterophagy Autophagy Lysosomal Phase . . . . . . . . . . . . . . . . . . . . . . . 28 Heterophagy Autophagy Post-lysosomal Phase . . . . . . . . . . . . . . . . . . . 28 Lysosomal Lipid Pigments Lysosomal Diseases . . . . . . . . . . . . . . . . . . . . 30 Delayed Enzyme Release Increased Enzyme Release Lysosomal Enzyme Deficiency Cytomembrane Lesions . . . . . . . . . . . . . . . 32 Apical Cell Edema Disrupted Communication Receptor Lesions . . . . . . . . . . . . . . . . . . . . . 32 Receptor dysfunction Cytoskeletal Lesions . . . . . . . . . . . . . . . . . . 34 Microtubular Lesions . . . . . . . . . . . . . . . . . . . 34 Polymerization Block Microfilament Lesions Intermediate Filament Lesions . . . . . . . . . . . 34 Actin Bodies Mallory Bodies 3 Connective Tissue Lesions Collagen Lesions . . . . . . . . . . . . . . . . . . . . . . 36 Types of Collagen . . . . . . . . . . . . . . . . . . . . . . 36 Biosynthesis Disorders . . . . . . . . . . . . . . . . . . 38 Ehlers-Danlos Syndrome (EDS) Scurvy Osteogenesis Imperfecta Fibrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40 Sequelae of Fibrosis . . . . . . . . . . . . . . . . . . . . 40 Pulmonary Fibrosis Sequelae of Fibrosis . . . . . . . . . . . . . . . . . . . . 42 Cirrhosis of the Liver Collagenolysis Disorders . . . . . . . . . . . . . . . . 44 a1-Antitrypsin Deficiency Fibrinoid Necrosis Collagen Hyalinization Collagen Vascular Diseases . . . . . . . . . . . . . . 46 Microfibril Lesions . . . . . . . . . . . . . . . . . . . . 48 Marfan’s Syndrome Amyloidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 48 General Definitions . . . . . . . . . . . . . . . . . . . . . 48 Structure of Amyloid . . . . . . . . . . . . . . . . . . . . 50 Types of Amyloid . . . . . . . . . . . . . . . . . . . . . . . 50 AL Amyloid AA Amyloid AF Amyloid AE Amyloid AS Amyloid Classification of Amyloidoses . . . . . . . . . . . . . 52 Systemic Amyloidosis Localized Amyloidosis Elastin Lesions . . . . . . . . . . . . . . . . . . . . . . . . 54 Biosynthesis Disorders . . . . . . . . . . . . . . . . . . 54 Restrictive Dermopathy Senile Elastosis Trichopoliodystrophy Type V Ehlers-Danlos Syndrome Elastosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56 Actinic Elastosis Pseudoxanthoma Elasticum Fibroelastosis . . . . . . . . . . . . . . . . . . . . . . . . . . 56 Sequelae of Fibroelastosis . . . . . . . . . . . . . . . . 56 Intimal Fibroelastosis Endocardial Fibroelastosis Riede / Werner, Color Atlas of Pathology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. Contents XIII Elastin Lysis Disorders . . . . . . . . . . . . . . . . . . 58 Sequelae of Elastin Lysis Disorders . . . . . . . . 58 Polyarteritis nodosa Temporal Arteritis Syphilitic Aortitis Proteoglycan Lesions . . . . . . . . . . . . . . . . . 60 Proteoglycan Complexing Disorders . . . . . . 60 Mucoid Degeneration Sequelae of Mucoid Degeneration . . . . . . . . 60 Mucoid Meniscopathy Ganglion Cystic Vascular Degeneration Mitral Valve Prolapse Syndrome Sequelae of Mucoid Degeneration . . . . . . . . 62 Proteoglycan Secretion Disorders . . . . . . . . 64 Cystic Fibrosis . . . . . . . . . . . . . . . . . . . . . . . . . . 64 Sequelae of Cystic Fibrosis . . . . . . . . . . . . . . . 64 Bronchiectasis Cystic fibrosis of the pancreas Meconium ileus Liver cirrhosis Proteoglycan Lysis Deficiencies . . . . . . . . . . 66 Mucopolysaccharidoses . . . . . . . . . . . . . . . . . . 66 Hurler’s Syndrome Hunter’s Syndrome Mucolipidoses . . . . . . . . . . . . . . . . . . . . . . . . . . 66 4 Errors of Metabolism: Inorganic Compounds Oxygen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68 Acute Generalized Hypoxia . . . . . . . . . . . . . . 68 Acute Local Hypoxia . . . . . . . . . . . . . . . . . . . . 69 Chronic Hypoxia . . . . . . . . . . . . . . . . . . . . . . . 69 Fatty Degeneration Organ Atrophy Interstitial Fibrosis Calcium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70 Hypocalcemia . . . . . . . . . . . . . . . . . . . . . . . . . 70 Hypercalcemia . . . . . . . . . . . . . . . . . . . . . . . . . 70 Iron . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72 Iron Deficiency . . . . . . . . . . . . . . . . . . . . . . . . 72 Absolute Iron Deficiency Relative Iron Deficiency Iron Overload . . . . . . . . . . . . . . . . . . . . . . . . . 72 Hemochromatosis Secondary Siderosis Copper . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74 Copper Deficiency . . . . . . . . . . . . . . . . . . . . . . 74 Chronic Copper Deficiency Trichopoliodystrophy Copper Overload . . . . . . . . . . . . . . . . . . . . . . . 74 Wilson’s Disease 5 Errors of Metabolism: Organic Compounds Carbohydrates . . . . . . . . . . . . . . . . . . . . . . . . 76 Glycogenoses . . . . . . . . . . . . . . . . . . . . . . . . . . 76 Type I Glycogenosis Type II Glycogenosis Type III Glycogenosis Carbohydrate Malabsorption . . . . . . . . . . . . 78 Lactose Intolerance Saccharose-Isomaltose Intolerance Glucose Synthesis Disorders . . . . . . . . . . . . . 78 Fructose-1,6-Diphosphatase Deficiency Hereditary Fructose Intolerance Deficient Carbohydrate Metabolism . . . . . . 78 Diabetes Mellitus . . . . . . . . . . . . . . . . . . . . . . . 78 Type I Diabetes mellitus Type II Diabetes mellitus Sequelae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 80 Galactosemia . . . . . . . . . . . . . . . . . . . . . . . . . . 80 Lipids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82 Fat Uptake Disorders . . . . . . . . . . . . . . . . . . . 82 Malabsorption Syndromes . . . . . . . . . . . . . . . 82 Disorders Associated with Malabsorption . . . 82 Gluten Enteropathy Whipple Disease Fatty Degeneration . . . . . . . . . . . . . . . . . . . . . 84 Dietary Steatosis Transport Steatosis Absorptive Steatosis Retention Steatosis Disorders of Fat Catabolism . . . . . . . . . . . . . 84 Starvation Obesity Ketonemia Phytanic Acid Storage Disease Propionic Acidemia Methylmalonic Aciduria Hypolipoproteinemia Disorders . . . . . . . . . . 86 Abetalipoproteinemia Analphalipoproteinemia Hyperlipoproteinemia Disorders . . . . . . . . . 88 Type I Hyperlipoproteinemia Type II Hyperlipoproteinemia Riede / Werner, Color Atlas of Pathology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. XIV Contents Type III Hyperlipoproteinemia Type IV Hyperlipoproteinemia Type V Hyperlipoproteinemia Familial LCAT Deficiency Sequelae of Hyperlipoproteinemia . . . . . . . . 90 Atherosclerosis Xanthoma Sphingolipidoses . . . . . . . . . . . . . . . . . . . . . . . 92 Gaucher’s Disease Sphingomyelin Lipidosis Krabbe’s Disease Metachromatic Leukodystrophy Fabry Disease Type I GM2 Gangliosidosis GM1 Gangliosidosis Proteins . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96 Defective Serum Proteins . . . . . . . . . . . . . . . 96 Disorders of Amino Acid Metabolism . . . . . 96 Phenylketonuria Albinism Alkaptonuria1 Maple Syrup Urine Disease Primary Hyperoxaluria Type I Homocystinuria Cystinuria Cystinosis Urea Synthesis Disorders . . . . . . . . . . . . . . 100 Nucleotides . . . . . . . . . . . . . . . . . . . . . . . . . 102 Hyperuricemia . . . . . . . . . . . . . . . . . . . . . . . . 102 Primary Gout Sequelae of Adult Gout Secondary Gout 6 Pigment Lesions Exogenous Pigments . . . . . . . . . . . . . . . . 104 Cosmetic Pigments . . . . . . . . . . . . . . . . . . . . 104 Occupational Toxin Pigments . . . . . . . . . . . 104 Anthracosis Plumbism Chalcosis Iatrogenic Pigments . . . . . . . . . . . . . . . . . . . 104 Argyria Amalgam Tetracycline Carotene Endogenous Pigments . . . . . . . . . . . . . . . 106 Hematogenous Pigments . . . . . . . . . . . . . . 106 Porphyria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 106 Congenital Erythropoietic Porphyria Hemoglobin Breakdown Pigments . . . . . . . 106 Hyperbilirubinemia . . . . . . . . . . . . . . . . . . . . . 108 Excessive Bilirubin Production Defective Bilirubin Uptake Defective Bilirubin Conjugation Defective Bilirubin Excretion Tyrosine-Derivative Pigments . . . . . . . . . . . . 112 Cutaneous Melanin . . . . . . . . . . . . . . . . . . . . . 112 Vitiligo Albinism Hemochromatosis Addison’s Disease Phenylketonuria Melasma Neuromelanin . . . . . . . . . . . . . . . . . . . . . . . . . . 114 Parkinson’s Disease Non-Melanin Pigments . . . . . . . . . . . . . . . . . . 114 Brown Colon Alkaptonuria Lipogenic Pigments . . . . . . . . . . . . . . . . . . . . 114 7 Sublethal Cell Damage Hypertrophy . . . . . . . . . . . . . . . . . . . . . . . . . 116 Types of Hypertrophy . . . . . . . . . . . . . . . . . . . 116 Diseases Associated with Hypertrophy . . . . . 116 Hypertrophic Cardiomyopathy Congenital Hypertrophic Pyloric Stenosis Hyperplasia . . . . . . . . . . . . . . . . . . . . . . . . . . 118 Types of Hyperplasia . . . . . . . . . . . . . . . . . . . . 118 Diseases Associated with Hyperplasia . . . . . . 118 Endometrial Hyperplasia Prostatic Hyperplasia Endocrinal Hyperplasia . . . . . . . . . . . . . . . . 120 Thyroid Hyperplasia Parathyroid Hyperplasia Hyperplasia of the Adrenal Cortex . . . . . . . 122 Adrenal Cortex Hyperfunction Syndrome . . 122 Hyperaldosteronism Hypercortisolism Adrenogenital Syndrome Atrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 Types of Atrophy . . . . . . . . . . . . . . . . . . . . . . . 124 Disorders AssociatedwithGeneralizedAtrophy 124 Senile Atrophy Atrophy Due to Inanition (Cachexia) Disorders Associated with Organ Atrophy . 126 Osteoporosis Alzheimer’s Disease Pick’s Disease Riede / Werner, Color Atlas of Pathology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. Contents XV 8 Lethal Cell Injury Apoptosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 128 Programmed Cell Death . . . . . . . . . . . . . 128 Commitment phase Executive phase Accidental Cell Death . . . . . . . . . . . . . . . . 130 Necrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 Focal Cytoplasmic Necrosis . . . . . . . . . . . . . . 130 Coagulative Necrosis . . . . . . . . . . . . . . . . . . . 130 Special Forms of Coagulative Necrosis . . . . 130 Liquefactive Necrosis . . . . . . . . . . . . . . . . . . 132 Special Forms of Liquefactive Necrosis . . . . 132 Shrinking Necrosis . . . . . . . . . . . . . . . . . . . . 132 Autolysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132 9 Calcification and Stone Disorders Calcification . . . . . . . . . . . . . . . . . . . . . . . . . 134 Dystrophic Calcification . . . . . . . . . . . . . . . . 134 Degenerative Calcification . . . . . . . . . . . . . . 134 Disorders Associated with Degenerative Calcification . . . . . . . . . . . . . . . . . . . . . . . . . . 134 Calcinosis cutis Myositis ossificans Valvular calcific degeneration Mo¨nckeberg medial calcific sclerosis Necrotic Calcification . . . . . . . . . . . . . . . . . . . 136 Metastatic Calcification . . . . . . . . . . . . . . . . 136 Disorders Associated with Metastatic Calcification . . . . . . . . . . . . . . . . . . . . . . . . . . 136 Pulmonary calcinosis Renal calcinosis Gastric calcinosis Myocardial calcinosis Stone Disorders . . . . . . . . . . . . . . . . . . . . . 138 Urolithiasis Cholelithiasis 10 Chemical Cell Injury Drug-Induced Disorders . . . . . . . . . . . . . 140 Drug-Induced Damage to Blood Cells . . . 140 Drug-Induced Skin Disease . . . . . . . . . . . . . 140 Drug-Induced Liver Disease . . . . . . . . . . . . . 141 Drug-Induced Kidney Disease . . . . . . . . . . . 141 Drug-Induced Lung Disease . . . . . . . . . . . . . 141 Drug-Induced Neurologic Disorders . . . . . . 141 Diseases from Environmental Factors 142 Carbon Monoxide Poisoning . . . . . . . . . . . 142 Sequelae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142 Severe carbon monoxide poisoning Acute carbon monoxide poisoning Lead Poisoning . . . . . . . . . . . . . . . . . . . . . . . 142 Sequelae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142 Metaphyseal lead lines Acquired porphyria Anemia Erythrocyte disease Lead nephropathy Lead encephalopathy Polyneuropathy Asbestosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142 Sequelae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142 Pneumoconiosis (asbestos lung) Pleural plaques Malignant mesotheliomas Disease from Alimentary Factors . . . . . 144 a-Amanitine Poisoning Alcoholism Sequelae of Alcoholism . . . . . . . . . . . . . . . . . 144 Alcoholic Fatty Degeneration of the Liver Alcoholic Hepatitis Alcoholic Liver Cirrhosis Megaloblastic Anemia Wernicke-Korsakoff Syndrome Cardiomyopathy 11 Physical Cell Injury Thermal Injury . . . . . . . . . . . . . . . . . . . . . . 146 Heat-Induced Injuries . . . . . . . . . . . . . . . . . . 146 Heat Stroke Burns Cold-Induced Injuries . . . . . . . . . . . . . . . . . . 148 Hypothermia Frostbite Sequelae of Cold Injuries . . . . . . . . . . . . . . . 148 Endangiitis obliterans Raynaud Syndrome . . . . . . . . . . . . . . . . . . . . 148 Riede / Werner, Color Atlas of Pathology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. XVI Contents Electrical Injuries . . . . . . . . . . . . . . . . . . . . 150 Radiation Injury . . . . . . . . . . . . . . . . . . . . . 150 Microwaves and Infrared Radiation . . . . . 150 Visible Light and Ultraviolet Radiation . . . 150 Sequelae of Light Radiation . . . . . . . . . . . . . 150 Acute photodermatosis Chronic photodermatosis Laser Radiation . . . . . . . . . . . . . . . . . . . . . . . 150 Ionizing Radiation . . . . . . . . . . . . . . . . . . . . . 152 Sequelae of Radiation . . . . . . . . . . . . . . . . . . 152 Radiation Vasculitis Radiation Dermatitis Radiation Enteritis Radiation Bone Necrosis Radiation-Induced Leukocyte Disease Radiation Therapy . . . . . . . . . . . . . . . . . . . . . 154 12 Immune Pathology Antigen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156 Antigen Neutralization . . . . . . . . . . . . . . . . 156 Immune Organs . . . . . . . . . . . . . . . . . . . . . . 157 Central Immune Organs . . . . . . . . . . . . . . . . 157 Thymus Bursa of Fabricius and Bone Marrow Peripheral Immune Organs . . . . . . . . . . . . . . 157 Lymph Nodes Spleen MALT SALT Cell-Mediated Immunity . . . . . . . . . . . . . . . 158 T Lymphocytes . . . . . . . . . . . . . . . . . . . . . . . . 158 Initial Immune Response . . . . . . . . . . . . . . . . 158 Effector Mechanisms Secondary Immune Response Humoral Immunity . . . . . . . . . . . . . . . . . . . . 160 B Lymphocytes . . . . . . . . . . . . . . . . . . . . . . . . 160 Initial Immune Response . . . . . . . . . . . . . . . . 160 Effector Mechanisms Secondary Immune Response . . . . . . . . . . . 162 Immune-Regulating Antigens . . . . . . . . . . . 164 Classification of the HLA Genes . . . . . . . . . . 164 Function of the HLA Antigens . . . . . . . . . . . 164 HLA-Associated Disorders . . . . . . . . . . . . . . . 164 Pathogenic Immune Reactions . . . . . . . 166 Hypersensitivity Reactions . . . . . . . . . . . . . 166 Type I Hypersensitivity Reactions . . . . . . . . . 166 Local Anaphylaxis Systemic Anaphylaxis Atopic Reaction Disorders Associated with Type I Hypersensitivity Reaction . . . . . . . . . . . . . . . 168 Bronchial Asthma Allergic Rhinosinusitis Type II Hypersensitivity Reactions . . . . . . . . 170 Type III Hypersensitivity Reactions . . . . . . . . 170 Serum Disease Disorders Associated with Serum Disease . 172 Immune Complex Vasculitis Immune Complex Nephritis Local Immune Complex Disease Disorders Associated with Local Immune Complex Disease . . . . . . . . . . . . . . . . . . . . . . . 174 Extrinsic Allergic Alveolitis Type IV Hypersensitivity Reaction . . . . . . . . . 174 Cutaneous Basophilic Hypersensitivity Reaction Contact Allergy Tuberculin Reaction Granulomatous Hypersensitivity Reaction Transplantation Pathology . . . . . . . . . . . . . . 178 Transplant Allergy Graft-Versus-Host Disease Autoimmune Disorders . . . . . . . . . . . . . . . . 180 Systemic Autoimmune Disorders . . . . . . . . . 180 Systemic Lupus Erythematosus Progressive systemic sclerosis Dermatomyositis Goodpasture’s Syndrome Wegener’s Granulomatosis Rheumatoid Arthritis Sjo¨gren’s Syndrome Autoimmune Disorders Organ specific . . . . 186 Hashimoto Thyroiditis Autoimmune Adrenalitis Type I Diabetes Mellitus Myasthenia Gravis Autoimmune Gastritis Multiple Sclerosis Immunodeficiency Disorders . . . . . . . . . . . 188 Primary Immunodeficiency Disorders . . . . . 188 X-Linked Agammaglobulinemia Transient Hypogammaglobulinemia Heavy-Chain Defect j Chain Defect IgG Subclass Defect Hyper IgM Syndrome Isolated IgA Deficiency Common Variable Immunodeficiency Primary Immunodeficiency Syndromes: T-Cell Defects . . . . . . . . . . . . . . . . . . . . . . . . . 189 DiGeorge Syndrome Nezelof Syndrome Chronic Myocutaneous Candidiasis Primary Immunodeficiency Syndromes: B-Cell and T-Cell Defects . . . . . . . . . . . . . . . . 190 Severe combined immunodeficiency (= SCID) Ataxia Telangiectasia Wiskott-Aldrich Syndrome Secondary Immunodeficiency Syndromes . 190 Monoclonal Gammopathy Acquired Immunodeficiency Syndrome (AIDS) Sequelae of AIDS . . . . . . . . . . . . . . . . . . . . . . 192 HIV Lymphadenopathy Kaposi’s Sarcoma HIV-Associated Pneumopathy HIV Encephalitis Riede /