Autoimmune & Inflammatory Disorders Flashcards
presence of antibodies (auto-antibodies or auto-reactive T-
cells) directed against normal host antigens (auto- or self-
antigens)
Autoimmunity
What are the autoantibodies seen in mucous membrane pemphigoid?
BP 180
BP 230
What are the autoantibodies seen in pemphigus vulgaris?
Demsoglein 1 and 3
Pathogenesis •Unknown etiology •Environmental triggers in a genetically predisposed individual •Chronic, inflammatory autoimmune disorder •Autoantibodies and immune complexes activate complement system •Vasculitis, fibrosis, tissue necrosis Complications Multi-organ Epidemiology •90% are young-middle aged women •2.5 times increased risk in AA
Systemic lupus erythematousus
What autoantibodies are present in SLE?
anti DS DNA and anti-SM
What other condition looks identical to SLE histologically and clinically intraorally?
Oral Lichen Planus
Which disease presents with a butterfly rash?
SLE
Management Rheumatologists: organ-specific approach •Long-term Prednisone •Immunomodulating agents (eg. mycophenolate mofetil, azathioprine, methotrexate) Oral Manifestations -lichenoid lesions •Ulcerations/erosions •Hard palatal mucosa ulcer •White radiating striae from a central ulcer
SLE
The following are dental considerations needing to be taken if pt has \_\_\_\_\_\_: Determine the status of the stability of their disease Leukopenia •Immunosuppressive rx Thrombocytopenia (25% of pts) •extreme thrombocytopenia (<20,000 platelets) Nonbacterial verrucous valvular Libman– Sacks endocarditis •most common cardiac lesion Renal disease •localization of immune complexes in the kidney is the precipitating factor in the development of lupus nephritis •rapidly progressing glomerulonephritis or a less aggressive form Neuropsychiatric disease •psychosis, seizures, cerebrovascular accidents
SLE
Pathogenesis •Unknown etiology •Environmental, hormonal, infectious factors, in a genetically predisposed individual •external trigger (infection, trauma) elicits an autoimmune reaction •hypertrophy of the synovial lining of the joint and endothelial cell activation •uncontrolled inflammation and destruction of cartilage and bone
Rheumatoid arthritis
•1.3 million US adults •Women 3 times more affected than men •sex difference diminish in older age groups, suggesting a hormonal component Diagnosis 6/10 score based on a 4-prong algorithm: 1. joint involvement 2. serology test results (Rheumatoid factor, ACPA anticitrullinated protein antibody) 3. acute-phase reactant test results (CRP, ESR) 4. pt self-reporting of signs/symptom duration
Rheumatoid arthritis
What lab value will be higher in Rheumatoid arthritis?
Rheumatoid factor
Management Nonpharmacological •Physical, occupational therapies, orthotic devices, surgery Pharmacological •NSAIDs, disease-modifying antirheumatic drugs, immunosuppressants, biological response modifiers, corticosteroids Oral Manifestations •TMJ dysfunction •Medication induced ulcerations •Salivary hypofunction Dental Considerations See ADA guideline on prophylactic antibiotics for pts with prosthetic joints undergoing dental procedures
Rheumatiod arthritis
Pathogenesis •Unknown etiology •Endothelial cell injury •Fibroblast activation •Vascular dysfunction Complications •skin thickening/induration •tissue fibrosis and chronic inflammation •infiltration of heart, lungs and kidneys •prominent fibroproliferative vascular disease Epidemiology •Peak onset (30–50 years) •4-9 times higher in women than in men
Progressive Systemic Sclerosis
Scleroderma
Diagnosis Localized scleroderma: •skin on the hands and face •slow disease course •indolent and rarely spreads more widely or results in serious complications Systemic scleroderma: •affects large areas of skin and heart, lungs, or kidneys 2 main types of systemic scleroderma: (1) limited disease or CREST syndrome, calcinosis, Raynaud’s syndrome, esophageal dysmotility, sclerodactyly, telangiectasia (2) diffuse disease
Systemic sclerosis
Management •Long-term Prednisone
•Immunomodulating agents (eg.
mycophenolate mofetil, azathioprine,
methotrexate)
Oral Manifestations Severe microstomia, trismus, submucosal
fibrosis
Dental Considerations Elongation exercises to improve trismus
Systemic sclerosis
Pathogenesis •Chronic, autoimmune, inflammatory disorder •Primary vs Secondary SS (RA, SLE) •Unknown etiology •expression of MHC-II molecules in activated salivary gland cells •inherited susceptibility markers trigger a chronic inflammatory response in genetically susceptible individuals •ongoing activation of the innate immune system as pro- inflammatory cytokines (eg. IFN-g) are elevated Complications •Lymphocytic infiltration •lacrimal glands causing dry eyes (xerophthalmia) •salivary glands causing drymouth (xerostomia)
Sjogren’s syndrome
Epidemiology •Elderly women •Female-to-male ratio is 9 : 1 Diagnosis SS ACR Classification Crietria (2/3) 1. Positive anti-Ro and/or anti-La or (+RF and ANA titer >1:320) 2. Minor labial salivary gland biopsy exhibiting focal lymphocytic sialadenitis focus score >1 focus/4mm2 3. Ocular staining score >3 Management •Rituximab (anti-CD20 monoclonal antibodies) •Long-term Prednisone •Immunomodulating agents (eg. mycophenolate mofetil, azathioprine, methotrexate) Dental Considerations Salivary hypofunction •stimulated and unstimulated salivary flow measurements •44 times increased risk of MALToma
Sjogren’s Syndrome
Deficiency of C1 esterase inhibitor so can’t inhibit bradykinin which results in increase vasodilation and vascular permeability
hereditary angioedema
\_\_\_\_\_ is idiopathic or immune mediated - angular chelitis -Lip swelling -Tongue fissuring -Cobblestoning -Uclers Ranges from segmental or diffuse erythema to edematous hyperplastic gingiva +/- pain Stag horn appearance -Median age 24 -Food sensitivity can trigger this Tx: -Diet modification •Topical/intralesional/systemic corticosteroids •delayed release triamcinolone •Various response •Adjuvant therapies •clofazimine, sulfasalazine, hydroxychloroquine, methotrexate, danazol, dapsone, TNF-alpha ant., metronidazole
Orofacial granulomatosis