Systemic Lupus Erythematosus
4000310515center4/2/201733000950004/2/2017420003263900880008851265Amanda Munz 450000Amanda Munz 420003263900175001760220Systemic Lupus ErythematosusCase Study450000Systemic Lupus ErythematosusCase StudyDate: 04/02/17Name: A.M. Gender: FemaleRace: CaucasianOccupation: Pharmacy technicianChief Complaint: “I think I am running a fever and my knees are hurting. I also have a rash on my face”Subjective: 24-year-old female presents with fever, butterfly rash, and joint pain.Differential DiagnosisClinical PresentationInfluenzaFever, cough, headache, sore throat, weakness, and fatigueChickenpoxSuperficial cutaneous lesions, fever, malaise, anorexiaRosaceaRed facial complexion that looks like a rash across the nose and cheeksMononucleosisFever, fatigue, general malaise, atypical lymphocytosisSystemic Lupus Erythematosus (lupus) is a chronic autoimmune disease that targets the tissues of the body and can affect multiple organ systems. It has a diverse array of clinical manifestations. It can affect people of all ages, ethnic groups, and sexes, but the vast majority of people presenting with lupus are women in their childbearing years. When considering a diagnosis of Systemic Lupus, other autoimmune diseases, infectious diseases, central nervous system diseases and fibromyalgia must also be considered because they also manifest with multisystem disease involvement. Clinical Presentation: Systemic Lupus can have several different presentations including constitutional, dermatologic, musculoskeletal, pulmonary, cardiac, vascular, renal, gastrointestinal, neuropsychiatric, neonatal Lupus, and drug-induced Lupus.Constitutional: Common constitutional symptoms include fever, fatigue, weight loss, myalgia and arthralgia.Dermatologic: Malar (butterfly) rash where the “body” of the butterfly is separate from the wings, and sometimes alopecia.Musculoskeletal: Pain and stiffness that is sometimes migratory and transient, periarticular inflammation, muscle disease, fibromyalgia symptoms, and myositis.Pulmonary: Serositis, shrinking lung, acute pneumonitis, alveolar hemorrhage, pulmonary hypertension, and thromboembolic-related disease.Cardiac: Occurs in 20-30% of patients and most common in Latin Americans. Pericardial effusions are seen in 20% of patients. Precodial chest pain that is worse lying down and relieved by leaning forward. Myocarditis, and coronary artery disease are also associated with systemic lupus. Vascular: Raynaud’s phenomenon occurs in 1/3 of patients at the onset of Systemic Lupus. Venous thromboembolism, and inflammatory vascular disease in the form of vasculitis can also occur. Renal: If renal disease presents in patients with lupus, there is a significant increase in morbidity. Permanent renal damage may occur. Gastrointestinal: Symptoms can occur in the oral cavity, esophagus, abdomen, intestines, pancreas, or liver. Neuropsychiatric: Neurologic manifestations can be primary or secondary manifestations and can either be organic or functional. These symptoms include seizures, CNS vasculitis, headaches, stroke, neuropathies, movement disorders, transverse myelitis, ophthalmic involvement, and meningitis. Psychiatric manifestations can include psychosis, and cognitive disorders.Neonatal Lupus: Occurs in 1-2% of babies, usually born to mothers with Systemic Lupus. Can have partial or complete heart blocks, bradycardia, and rash on scalp and periorbital area. Drug-Induced Lupus: Epidemiologically, clinically and serologically different from systemic lupus. Most commonly associated with isoniazid, hydralazine, procainamide, minocycline, aldomet, diltiazem, penicillamine, infliximab, etanercept, rifampin, quinidine, captopril, beta blockers, anticonvulsants, sulfa, and amiodarone. DIAGNOSTIC TESTINGThere is no single test to diagnose lupus. The “gold standard” for diagnosing lupus is the “eleven criteria” which is; Malar rash, discoid rash, photosensitivity, mouth or nose ulcers, arthritis, cardio-pulmonary involvement, neurologic disorder, renal disorder, hematologic disorder, immunologic disorder, and antinuclear antibodies. A positive diagnosis of Systemic Lupus only requires 4 of the 11 criteria to be present, but not all patients will fulfill these criteria. TESTRESULT FOR A POSITIVE DIAGNOSIS FOR SLECBC plus differentialAnemia, thrombocytophenia, leukopenia, lymphopenia, occasional neutropeniaBasic metabolic panelElevated BUN/Cr ratioESR and CRPElevatedComplements (C3 and C4)LowUrine ChemistryProteinuria, hematuria, RBCs, red cell and mixed castsLFT’sElevated transaminases and/or alkaline phosphatase, low protein or albumindsDNA antibodyPositive; higher titers seem to predict disease severity at timesAPLAsLupus anticoagulant panel, cardiolipin antibody panel, beta2 glycoproteinPATHOPHYSIOLOGYSystemic Lupus Erythematosus is a complex array of immune system abnormalities. There is some evidence that heredity plays a role in the development of the disease. There are many abnormalities in T cell function, B cell function, programmed cell death, immune complex clearance, complement function and deficiencies, and nucleus processing. It is not certain what starts the immune dysregulation. Different ethnic groups have different genetic abnormalities. Most of the pathology of lupus is related to immune complex which deposit in various organs triggering complement and other mediators of inflammation. TREATMENTTreatment options usually include steroids, hydroxychloroquine, dapsone, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide, and rituximab. Because each case of lupus is different for each patient, treatment is usually designed around the patient being treated. Osteoporosis prevention and treatment is vital for patients taking steroids. PROGNOSISThe mortality rate of patients with SLE is 3 times higher than the general population and even worse in African American patients, patients with early-onset disease, and in patients with renal, cardiopulmonary and CNS involvement and the overall prognosis has improved since the 1950s due to improved diagnostic techniques. REFERENCESBartels, C. (2016). Systemic Lupus Erythematosus (SLE) Clinical Presentation. Retrieved from , M. Drzymkowski, J. (2016). Essentials of Human Diseases and Conditions. Canada: Elsevier, Inc. Pathol, J. (2003). Pathogenesis of systemic lupus erythematosus. Retrieved from , B. Bharadwaj, S. (2010). Systemic Lupus Erythematosus. Retrieved from from (2016). Lupus Diagnosis. Retrieved from ................
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