Rheumatological Rheumatological Diseases Diseases Rheumatological - PDF document

Rheumatologic Diseases Rheumatologic Diseases 6/23/2011 6/23/2011 Rheumatological Rheumatological Diseases Diseases Rheumatological Rheumatological Diseases Diseases Disease Disease Autoantibody Autoantibody Systemic Lupus Erythematosus Anti- Systemic Lupus Erythematosus Anti -dsDNA, Anti dsDNA, Anti- -SM SM Rheumatoid Arthritis Rheumatoid Arthritis RF, Anti RF, Anti- , -RA33 RA33 Sjogrens Syndrome Sjogrens Syndrome Anti- Anti -Ro(SS Ro(SS- -A),Anti A),Anti- -La(SS La(SS- -B) B) Fernando Vega, MD Fernando Vega, MD Systemic Sclerosis Systemic Sclerosis Anti- Anti -Scl Scl- -70, Anti 70, Anti- -centromere centromere HIHIM 409 HIHIM 409 Polymyositis/Dermatomyositis Polymyositis/Dermatomyositis Anti Anti- -Jo Jo- -1 1 Mixed Connective Tissue Disease Anti- Mixed Connective Tissue Disease Anti -U1 U1- -RNP RNP July 2, 2009 July 2, 2009 Wegener’s Granulomatosus Wegener’s Granulomatosus c- c -ANCA ANCA Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus � General General � Common complaints and presentations Common complaints and presentations � � autoimmune multisystem disease autoimmune multisystem disease � Fever Fever � prevalence 1 in 2,000 � prevalence 1 in 2,000 prevalence 1 in 2,000 prevalence 1 in 2,000 � Malaise � Malaise Malaise Malaise � 9 to 1; female to male (1 in 700) 9 to 1; female to male (1 in 700) � Joint pains Joint pains � peak age 15 peak age 15- -25 25 � Myalgias Myalgias � immune complex immune complex deposition deposition � Fatigue Fatigue � Loss of cognitive abilities Loss of cognitive abilities Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus � Dermatological Manifestations Dermatological Manifestations � 40 40- -60 % present with some 60 % present with some derm derm symptom symptom � 30 � 30 30 30 – 60 % of them as a 60 % of them as a malar 60 % of them as a malar 60 % of them as a malar rash malar rash rash rash � Photodermatitis Photodermatitis � Discoid lupus Discoid lupus - - � Alopecia Alopecia � Mucosal ulcers Mucosal ulcers Fernando Vega, M.D. Fernando Vega, M.D. 1

Rheumatologic Diseases Rheumatologic Diseases 6/23/2011 6/23/2011 Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus � Discoid Lupus: Cutaneous manifestations Discoid Lupus: Cutaneous manifestations � Scar upon healing Scar upon healing Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Systemic Lupus Erythematosus Erythematosus � Musculoskeletal Manifestations Musculoskeletal Manifestations � Joint pains Joint pains � Often small joints Often small joints – wrist and fingers j wrist and fingers g � Not as disabling as RA of hand and wrist Not as disabling as RA of hand and wrist � Doesn’t cause severe destruction of joints Doesn’t cause severe destruction of joints Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Systemic Lupus Erythematosus Erythematosus End Organ Damage End Organ Damage � Most common cause of death is end organ Most common cause of death is end organ � Musculoskeletal 25.2% Musculoskeletal 25.2% damage damage � Neuropsychiatric 15% Neuropsychiatric 15% � 80% of end organ damage is caused by � 80% of end organ damage is caused by 80% of end organ damage is caused by 80% of end organ damage is caused by � Ocular 12 6% � Ocular 12.6% Ocular 12.6% Ocular 12 6% Prednisone Prednisone � Renal 11.7% Renal 11.7% � Pulmonary 10.4% Pulmonary 10.4% � Cardiovascular 10.1% Cardiovascular 10.1% � Gastrointestinal 7.3% Gastrointestinal 7.3% � Skin 7.4% Skin 7.4% � Peripheral Peripheral Vascular 5.5% Vascular 5.5% � Diabetes Mellitus Diabetes Mellitus Fernando Vega, M.D. Fernando Vega, M.D. 2

Rheumatologic Diseases Rheumatologic Diseases 6/23/2011 6/23/2011 Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus Exogenous Exogenous Estorogens Estorogens Exogenous Estrogens Exogenous Estrogens SELENA Study SELENA Study � Estrogen increases autoantibody production Estrogen increases autoantibody production � Hormone Replacement Therapy � Hormone Replacement Therapy Hormone Replacement Therapy Hormone Replacement Therapy � 2 alpha 2 alpha vs 2 2 l h l h vs 16 alpha hydroxylation 16 16 alpha hydroxylation 16 l h l h h d h d l ti l ti � No increase in severe flares No increase in severe flares � Reversing hydroxylation pathway Reversing hydroxylation pathway � 20%increse in overall flares ( 20% increse in overall flares (Lahita Lahita 1981) 1981) � Broccoli helps reverse hydroxylation (clinically Broccoli helps reverse hydroxylation (clinically � Oral Contraceptive Therapy Oral Contraceptive Therapy relevant?) relevant?) � No increase in flares No increase in flares But 50% of women have But 50% of women have aPL aPL Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus Systemic Lupus Erythematosus Environmental Triggers Environmental Triggers Prognosis and Survival Prognosis and Survival 100% 100% 1953- 1953 -1978 1978 � UVA and UVB UVA and UVB 1978 1978 90% 90% � Drugs: Drugs: Bactrim g Bactrim and and Neupogen Neupogen p g p g 80% 80% � Smoking Smoking � Infections Infections 1960 1960 70% 70% � Pets Dogs Pets Dogs � � Lab workers Lab workers � 60% 60% 1953 1953 � Silica Silica 50% 50% � Mercury Mercury 0 1 1 2 2 3 3 4 4 5 Anti Anti- -Phospholipid Phospholipid Syndrome Syndrome Anti- Anti -Phospholipid Phospholipid Syndrome Syndrome Definition: Definition: � APS occurs either as a primary condition or as a APS occurs either as a primary condition or as a � Presence in the serum of at least one type of Presence in the serum of at least one type of secondary condition in the setting of an underlying secondary condition in the setting of an underlying autoantibody known as an autoantibody known as an antiphospholipid antiphospholipid antibody antibody (aPL (aPL aPL) aPL) disease, particularly disorders in the spectrum of disease particularly disorders in the spectrum of disease particularly disorders in the spectrum of disease, particularly disorders in the spectrum of systemic lupus systemic lupus erythematosus erythematosus (SLE). (SLE). � The occurrence of at least one clinical feature from a The occurrence of at least one clinical feature from a diverse list of potential disease manifestations, the most diverse list of potential disease manifestations, the most common: common: � venous or arterial thromboses venous or arterial thromboses � recurrent fetal loss recurrent fetal loss � thrombocytopenia thrombocytopenia Fernando Vega, M.D. Fernando Vega, M.D. 3