Summary Head and Neck Manifestations of Systemic Disease

Section 1 Systemic Disorders with Head and Neck Manifestations Part A & Rheumatic Diseases Chapter 1 & Systemic Lupus Erythematosus, Scleroderma, Dermatomyositis/Polymyositis, and Rheumatoid Arthritis Chapter 2 & Sjögren’s Syndrome Chapter 3 & Adamantiades-Behçet’s Disease Chapter 4 & Giant Cell Arteritis and Polymyalgia Rheumatica Chapter 5 & Autoimmune Inner Ear Disease Chapter 6 & Uveitis, Cogan’s Syndrome, and Sarcoidosis Chapter 7 & Relapsing Polychondritis Chapter 8 & Granulomatous Diseases: Wegener’s Granulomatosis, Churg-Strauss Syndrome, and Nasal Natural Killer (NK)/T-Cell Lymphoma Chapter 9 & Kawasaki Disease Part B & Infectious Diseases Chapter 10 & Epstein-Barr, Herpes Simplex, and Herpes Zoster Infections Chapter 11 & Bacterial Diseases: Diphtheria, Cat-Scratch Disease, Gonorrhea, and Necrotizing Fasciitis Chapter 12 & Tuberculosis and Atypical Mycobacteria Chapter 13 & Fungal Rhinosinusitis Chapter 14 & Mucormycosis Chapter 15 & Syphilis Chapter 16 & Human Immunodeficiency Virus and Acquired Immune Deficiency Syndrome Part C & Malignant Diseases Chapter 17 & Lymphoma, Myeloproliferative Disorders, and Leukemia Chapter 18 & Malignant Melanoma Chapter 19 & Histiocytosis, Sickle Cell Anemia, and Rhabdomyosarcoma Part D & Other Systemic Disorders Chapter 20 & Crohn’s Disease and Ulcerative Colitis Chapter 21 & Paget’s Disease of Bone and Fibrous Dysplasia Chapter 22 & Systemic Allergic Disorders: IgE-Mediated Disorders, Angioedema, Nonsteroidal Anti-Inflammatory Drug Sensitivity, and StevensJohnson Syndrome Chapter 23 & Migraine Syndrome Chapter 24 & Pseudotumor Cerebri and Jugular Foramen Syndrome Part A & Rheumatic Diseases 1 Systemic Lupus Erythematosus, Scleroderma, Dermatomyositis/ Polymyositis, and Rheumatoid Arthritis Sharon L. Kolasinski Department of Medicine, Division of Rheumatology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, U.S.A. & Introduction 4 & Definition 4 & Epidemiology 4 & Pathogenesis 5 & Clinical Manifestations 6 & Treatment 11 & Complications, Prognosis, and Outcome 12 & Summary 12 & References 12 3 INTRODUCTION The presentation and expression of multisystem disease is diverse. The onset of systemic diseases such as systemic lupus erythematosus (SLE), scleroderma, dermatomyositis (DM), polymyositis (PM), and rheumatoid arthritis (RA) may occur with symptoms in a variety of organ systems, sometimes typical and sometimes unusual. The eventual course of the disease may include involvement of yet other organ systems. While otorhinolaryngeal manifestations of systemic rheumatic diseases are not among the most common hallmarks of these diseases, they can be quite characteristic and their recognition and management can be of considerable importance (1). DEFINITION Systemic Lupus Erythematosus SLE is the prototypical autoimmune disease characterized by the production of numerous autoantibodies and a wide variety of clinical manifestations. Features of SLE that are of diagnostic importance occur in the head and neck regions regularly. These include abnormalities of the skin and mucous membranes. Other disease features include arthritis and renal and central nervous system abnormalities. Scleroderma Scleroderma can involve limited or widespread changes in the skin, with a host of associated internal organ manifestations. In case