“Systemic Lupus Erythematosus” Other clinical features Iraj Salehi-Abari “Systemic Lupus Erythematosus” Other clinical features Iraj Salehi-Abari MD., Internist Rheumatologist salehiabari@sina.tums.ac.ir SLE

Definition: Chronic disease Systemic/Multi-organ disease Iraj Salehi-Abari Definition: Chronic disease Systemic/Multi-organ disease Inflammatory/Autoimmune disease Autoantibody/Immune Complex Skin, Joints, Kidneys, Lungs, Nervous system, Serous membranes,… y SLE

Gastrointestinal features: Iraj Salehi-Abari Gastrointestinal features: Most commonly due to drugs Abdominal pain: 4P/H Peptic ulcer Peritonitis (SBP) Pancreatitis (SLE, Steroids, AZT) Perforation (mesentric vasculitis or thrombosis) Hepatitis SLE

Gastrointestinal features: Iraj Salehi-Abari Gastrointestinal features: Dysphagia: the most common GI complaint Gastroesophageal reflux (GERD) Dyspepsia Hepatosplenomegaly: 30-50% “Masked acute surgical abdomen” Protein-losing enteropathy SLE

Hepatitis (elevated liver enzymes): Iraj Salehi-Abari Hepatitis (elevated liver enzymes): “Lupus hepatitis” (SLE itself) NSAIDs hepatitis Viral hepatitis “Lupoid hepatitis” (autoimmune hepatitis): Type I (Classic): ANA &/or ASMA and AAA Type II: ALKM-1 &/or ALC-1 SLE

Pulmonary involvements: Iraj Salehi-Abari Pulmonary involvements: Occur in most patients Late onset SLE Pleural involvement Pleuritic chest pain: Chest wall pain > pleuritis Pleural effusion: Pleural friction rub SLE

Pulmonary involvements: Iraj Salehi-Abari Pulmonary involvements: Pleural effusion: Usually small to moderate Evanescent and recurrent Often bilateral Mild exudate: Clear, low Pr, Nl glucose, WBC < 5000, positive ANA, high Ics, high LDH SLE

Pulmonary involvements: Iraj Salehi-Abari Pulmonary involvements: Acute Lupus Pneumonitis (ALP): Uncommon with poor prognosis At first Bacterial pneumonia (> ALP) must be ruled out by: I. HRCT, II. BAL, III. Video-assisted thoracoscopic biopsy, IV. Open lung biopsy Fulminant hemorrhagic lupus pneumonitis: rare, letal SLE

Pulmonary involvements: Iraj Salehi-Abari Pulmonary involvements: Interstitial Lung disease (ILD) Pulmonary Artery Hypertension (PAH) Pulmonary Thrombo-Embolism (PTE) Shrinking (Vanishing) Lung syndrome (SLS) SLE

Pulmonary involvements: Iraj Salehi-Abari Pulmonary involvements: Bronchiolitis Obliterns with Organizing Pneumonia (BOOP) Acute reversible hypoxemia Adult respiratory distress syndrome (ARDS) SLE

Pulmonary involvements: Iraj Salehi-Abari Pulmonary involvements: SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Non-Coronary Heart disease Coronary Heart disease Due to I. SLE, II. APS, III. Drugs,IV. others SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Non-Coronary Heart disease Pericarditis Myocarditis Endocarditis (+ Valvular disease) Conduction Abnormality SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Pericarditis: One-half of cases Pericardial chest pain Pericardial friction rub Pericardial Effusion (Echo) ECG changes Pericardiocentesis SLE

Attention please: Tamponate (large PE) is rare in SLE Iraj Salehi-Abari Attention please: Tamponate (large PE) is rare in SLE Constrictive pericarditis is rare too Intractable PE : Purulent TB Neoplastic SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Myocarditis: uncommon/dangerous Heart Failure Conduction abnormalities Arrhythmias SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Myocarditis should be suspected if there are unexplained: Resting tachycardia ST-T-U abnormalities in ECG Cardiomegaly SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Cardiomyopathy (CMP) in SLE may be due to: “Lupus Myocarditis” Drug-induced CMP Uremia CMP Postpartum CMP SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Endocarditis (“Libman-Sacks”): Nonbacterial thrombotic/verrucous Valve thickening, nodules Valvular regurgitation/stenosis SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Endocarditis (“Libman-Sacks”): Heart Failure Embolic disease New or changing heart murmur: MAT Bacterial Endocarditis (SBE) SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Valvular disease (M., A., T.): Libman-Sacks endocarditis Antiphospholipid syndrome (APS): 50% Mitral valve prolapse (MVP): 25% Others SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Coronary heart disease Coronary Vasculitis Coronary spasm (Raynaud’s Ph.) Coronary Atherosclerosis Coronary thrombosis/embolism SLE

Cardiac manifestations: Iraj Salehi-Abari Cardiac manifestations: Coronary heart disease Angina pectoris/Unstable angina Myocardial infarction Prinzmetal angina Atypical Ischemic heart disease Asymptomatic SLE

Vascular involvements: Iraj Salehi-Abari Vascular involvements: Vasculitis Atherosclerosis Raynaud’s phenomenon Vascular thrombosis/embolism SLE

Vascular involvements: Iraj Salehi-Abari Vascular involvements: Vasculitis Leukocytoclasti vasculitis (LCV) Polyarteritis nodosa (PAN) Livedoid vasculitis Others SLE

Vascular skin lesions of SLE: Iraj Salehi-Abari Vascular skin lesions of SLE: Periungual erythema and telangiectasia Livedo reticularis Facial telangiectasia Raynaud phenomenon Petechia/purpura SLE

Vascular skin lesions of SLE: Iraj Salehi-Abari Vascular skin lesions of SLE: Microinfarcts of the tips of fingers and toes Splinter hemorrhages “Janeway lesion” “Osler’s nodes” Atrophie blanche Chilblain Lupus Erythematosus (CHLE) SLE

Neurologic manifestations: Iraj Salehi-Abari Neurologic manifestations: Cognitive defects: “Mild cognitive dysfunction” is the most common CNS feature of SLE Acute confusional state Dementia/Delirium Seizures Headache SLE

Neurologic manifestations: Iraj Salehi-Abari Neurologic manifestations: Cerebrovascular accident (CVA) Mononeuritis multiplex Peripheral/Cranial neuropathy Myelitis (transverse) Meningitis Movement disorders SLE

Neurologic manifestations: Iraj Salehi-Abari Neurologic manifestations: Multiple sclerosis (MS) like features: Clinical mimicry Lesions similar to MS plaques in MRI SLE  MS like feature MS  SLE like feature: “Lupoid Sclerosis”: MS + SLE: Overlap syndrome SLE

Neurologic manifestations: Iraj Salehi-Abari Neurologic manifestations: Electro-encephalography (EEG): 70% Lumbar puncture (LP): Elevated Protein: 50% Elevated cell: 30% Brain MRI: the most sensitive imaging SLE

Psychologic manifestations: Iraj Salehi-Abari Psychologic manifestations: Psychosis Depression Anxiety Mania Bipolar disorder SLE

Psychologic manifestations: Iraj Salehi-Abari Psychologic manifestations: Personality disorder Emotional lability Sexual indiscretions Verbosity Religiosity Aggressiveness SLE

Ophthalmologic manifestations: Iraj Salehi-Abari Ophthalmologic manifestations: Keratoconjunctivitis sicca: most common Retinal vasculitis: dangerous Episcleritis or scleritis Anterior Uveites SLE

Hematologic manifestations: Iraj Salehi-Abari Hematologic manifestations: Cytopenia: Leukopenia Anemia: AOCD > Hemolytic anemia Thrombocytopenia Thrombophilia SLE

Hematologic manifestations: Iraj Salehi-Abari Hematologic manifestations: Leukopenia: WBC < 4500/mm3: leukopenia WBC < 4000/mm3: criteria WBC < 2000/mm3: severe/symptomatic SLE

Hematologic manifestations: Iraj Salehi-Abari Hematologic manifestations: Anemia: Hb < 12 in women, Hb < 14 in men Anemia of chronic disease (AOCD): most common “Hemolytic anemia”: rare, criteria (+) Commb’s test  Immune HA (-) Commb’s test  non-Immune HA SLE

Hematologic manifestations: Iraj Salehi-Abari Hematologic manifestations: Thrombocytopenia Platelet counts <150,000/mm3 Platelet < 100,000: criteria Platelet < 25,000  bleeding SLE

Hematologic manifestations: Iraj Salehi-Abari Hematologic manifestations: “Evan’s syndrome”: Immune thrombocytopenic purpura (ITP) along with Immune hemolytic anemia Highly suggestive of SLE SLE

Thrombophilia: Etiology of Thromboembolic disease: Iraj Salehi-Abari Thrombophilia: Etiology of Thromboembolic disease: Antiphospholipid syndrome Nephrotic syndrome: DVT, RVT Protein C, S, and antithrombin III deficiency Vasculitis SLE

Thrombophilia: Clinical syndromes: Antiphospholipid syndrome (APS) Iraj Salehi-Abari Thrombophilia: Clinical syndromes: Antiphospholipid syndrome (APS) Thrombotic thrombocytopenic purpura (TTP) Hemolytic uremic syndrome (HUS) Disseminated intravascular coagulation (DIC) SLE

Lymphadenopathy: 50% of cases of SLE The onset of disease/exacerbation Iraj Salehi-Abari Lymphadenopathy: 50% of cases of SLE The onset of disease/exacerbation Other causes: Infections Lymphoma: NHL, angioimmunoblastic T cell L Lymph node biopsy: Disproportionately enlarged, no response to medium to high doses of steroids SLE

Antiphospholipid syndrome (APS): Iraj Salehi-Abari Antiphospholipid syndrome (APS): Clinical criteria: Vascular thrombosis Pregnancy morbidity: At least one Unexplained fetal death at > 10 weeks gestation or At least one premature births before 34 weeks of gestation or At least three abortion before 10 weeks of gestation SLE

Antiphospholipid syndrome (APS): Iraj Salehi-Abari Antiphospholipid syndrome (APS): Laboratory (aPL) criteria: Anti-cardiolipin (aCL): IgG/IgM; moderate to high Anti-B2 Glycoprotein I (anti-B2 GPI); Lupus anticoagulant (LA) SLE

Antiphospholipid syndrome (APS): Iraj Salehi-Abari Antiphospholipid syndrome (APS): Revised “Sapporo” criteria: At least one clinical criteria along with one aPL The aPL antibody must be positive on two or more occasions at least 12 weeks apart and no more than 5 years prior to clinical criteria SLE

SLE in Men Higher frequencies of…, than women Renal, Skin, Cytopenias Iraj Salehi-Abari SLE in Men Higher frequencies of…, than women Renal, Skin, Cytopenias Serositis Neurologic involvement Thrombosis Cardiovascular, HTN and vasculitis Lower frequencies of …, than women Raynaud phenomenon Photosensitivity Mucosal ulceration SLE

Iraj Salehi-Abari SLE in Children: More severe than in adults with higher frequencies of: Malar rash Nephritis Pericarditis Hepatosplenomegaly Hematologic abnormalities SLE

SLE in Older adults: Milder than adults with lower incidence of: Iraj Salehi-Abari SLE in Older adults: Milder than adults with lower incidence of: Malar rash, photosensitivity Purpura, alopecia,RP Nephritis, CNS lupus and Hematologic manifestations Anti-RO, anti-Sm, anti-RNP Higher incidence of Sicca symptoms,serositis Pulmonary involvement Musculoskeletal manifestations Rheumatoid factor (RF) SLE

Initial presentation: Iraj Salehi-Abari Initial presentation: Fever, fatigue, or weight loss Malar rash, photosensitivity Arthralgia/myalgia or arthritis Nephritic and/or nephrotic syndrome pleuritis and/or pericarditis Alopecia DLE/SCLE SLE

Initial presentation: Iraj Salehi-Abari Initial presentation: Anemia/leukopenia/thrombocytopenia Seizure and/or psychosis MI and/or CVA without any risk factor in young ladies APS features Vasculitis: LCV, PAN in young ladies, Urticarial vasculitis Oral ulcer and/or pharyngitis Raynaud phenomenon SLE

The most common pattern: Iraj Salehi-Abari The most common pattern: Women in child-bearing ages with Constitutional complaints and Skin Musculoskeletal Hematologic and serologic involvement SLE

Classic case: Female within childbearing age Iraj Salehi-Abari Classic case: Female within childbearing age Fatigue, malaise, weight loss Polyarthralgia, myalgia, polyarthritis Malar rash with photosensitivity Alopecia, oral ulcer Anemia, Leukopenia Depression/Anxiety Positive FANA SLE