Systemic lupus erythematosus - Chronic
Rahul Soman, M. Pharm
Definition
A chronically remitting and relapsing, inflammatory, multisystemic autoimmune disorder primarily affecting women of childbearing age .
Ongoing Assessment
Clinical Evaluation
Renal Disease
Renal biopsy findings help guide pSLE management, especially decisions pertaining to low- or high-dose corticosteroid and immunosuppressant therapies.
* Kidney biopsy
Hematologic involvement
Suspected autoimmune hemolytic anemia
* Direct Coombs test: A positive direct Coombs test requires further analysis of IgG or complement on red blood cells to determine the hemolytic mechanism .
Suspected or known systemic lupus erythematosus
* Complete blood count: Hematologic abnormalities (anemia, leukopenia, and/or thrombocytopenia) are characteristic of active systemic lupus erythematosus (SLE) .
Cardiac Involvement
Suspected pericarditis
* 12 lead ECG: The ECG is an essential tool for evaluating acute pericarditis, and is helpful in assessing constrictive pericarditis and cardiac tamponade .
Endocrine involvement
Suspected diabetes mellitus
* Plasma fasting glucose measurement: Fasting plasma glucose levels of 126 mg/dL (7 mmol/L) or greater are diagnostic of diabetes mellitus. In the absence of unequivocal hyperglycemia, results must be confirmed with repeat testing on another day .
Suspected or known hypothyroidism
* Thyroid stimulating hormone measurement: Overt hypothyroidism is classified as a TSH above the upper limit of the reference range in the presence of a low serum free thyroxine (FT4) . Subclinical hypothyroidism is a laboratory diagnosis defined as a TSH above the upper limit of the reference range in the presence of a normal FT4.
Management Classification
Renal Biopsy-Based Management Classification in Lupus Nephritis
Renal biopsy findings help guide pSLE management, especially decisions pertaining to low- or high-dose corticosteroid and immunosuppressant therapy.
* Kidney biopsy
Suspected and known chronic kidney disease
* Creatinine measurement, serum: Serum creatinine levels should be obtained in all patients with known or suspected chronic kidney disease to estimate the GFR .
Suspected and known lupus nephritis
* Urinalysis: In systemic lupus erythematosus, urinalysis findings of hematuria, casts, pyuria, and/or proteinuria indicate lupus nephritis.
Medical History
* Female
* Family history of Systemic lupus erythematosus
* Infectious disease
* Occupational Exposure
* Environmental Exposure
* Stress
Findings
* Headache
* Impaired cognition
* Mental state finding
* Pleurisy
* Breath sounds - finding
* Cramping pain
* Cranial nerve disorder
* Dry cough
* Dyspnea - Chronic
* Hemoptysis
* Idiopathic livedo reticularis
* Lupus vasculitis
* Myocarditis
* Pericarditis secondary to systemic lupus erythematosus
* Peripheral neuropathy - Chronic
* Psychotic disorder
* Retinal vasculitis
* Seizure
* Severe visual impairment
* Venous thrombosis, phlebitis and thrombophlebitis
* Abnormal weight gain
* Alopecia due to underlying disease
* Arthralgia
* Bleeding gums
* Butterfly rash
* Discoid lupus erythematosus
* Edema of eyelid
* Fever
* Has nosebleeds - epistaxis
* Jaccoud's syndrome
* Joint swelling
* Keratoconjunctivitis sicca
* Loss of appetite
* Lymphadenopathy
* Malaise and fatigue
* Mucosal ulcer
* Myalgia
* Nausea, vomiting and diarrhea
* Photosensitivity
* Pitting edema
* Subacute cutaneous lupus erythematosus
* Systemic lupus erythematosus arthritis
* Weight loss
* Chorea in systemic lupus erythematosus
* Raynaud's phenomenon
Tests
Suspected or known systemic lupus erythematosus
* Complete blood count: Hematologic abnormalities (anemia, leukopenia, and/or thrombocytopenia) are characteristic of active systemic lupus erythematosus (SLE) .
Suspected systemic lupus erythematosus
* Antibody to SM measurement: A positive result on a Smith antibody assay usually confirms diagnosis of systemic lupus erythematosus, but a negative result does not exclude the diagnosis .
Suspected systemic lupus erythematosus
* Antibody to double stranded DNA measurement: High titers of anti-dsDNA antibodies are nearly pathognomonic for SLE, especially if coincident with other autoantibodies such as anti-Sm .
Suspected systemic lupus erythematosus
* ANA measurement: Antinuclear antibodies are detected in 98% to 100% of patients with systemic lupus erythematosus .
Suspected and known lupus nephritis
* Urinalysis: In systemic lupus erythematosus, urinalysis findings of hematuria, casts, pyuria, and/or proteinuria indicate lupus nephritis.
Suspected C3 deficiency
* C3 complement assay: Immune complex diseases such as systemic lupus erythematosus (SLE) have been associated with decreased levels of complement C3 .
Suspected C4 deficiency
* C4 complement assay: C4 deficiency is associated with certain nephritic autoimmune diseases .
Suspected systemic lupus erythematosus
* Antibody to SS-A measurement: A positive anti-Ro/SSA test is highly indicative of SLE and may help confirm the diagnosis in a patient with otherwise uncertain findings .
Suspected systemic lupus erythematosus
* Antibody to SS-B measurement: A positive titer on an anti-SS-B antibody test means that a patient may be 40 times more likely than controls to develop systemic lupus erythematosus .
Guideline recommendation to assist in diagnosis of SLE and as baseline for monitoring disease activity .
* Erythrocyte sedimentation rate measurement
Suspected and known chronic kidney disease
* Creatinine measurement, serum: Serum creatinine levels should be obtained in all patients with known or suspected chronic kidney disease to estimate the GFR .
Differential Diagnosis
* Rheumatoid arthritis
* Drug-induced lupus erythematosus
* Immune thrombocytopenic purpura
* Wegener's granulomatosis
* Primary antiphospholipid syndrome
* Mixed connective tissue disease
* Primary Sjögren's syndrome
* Systemic sclerosis
* Polymyalgia rheumatica
* Primary fibromyalgia syndrome
Treatment
Drug Therapy
Mild systemic lupus erythematosus (SLE)
IBUPROFEN (Related toxicological information in IBUPROFEN)
Adults: 600 mg orally 3 times daily; total dosage not to exceed 3.2 g/day
ASPIRIN (Related toxicological information in SALICYLATES)
Adults: 650 mg orally every 4 hours; total dosage not to exceed 3.6 g/day
HYDROXYCHLOROQUINE SULFATE
Adults: 200 mg orally 1 or 2 times daily (indefinitely)
Pediatrics: 5 mg/kg orally daily; not to exceed 400 mg daily
PREDNISONE
Adults: Up to 10 mg orally daily; may use alternate day dosing
Betamethasone Dipropionate
Adults: Thin film applied to skin lesions 1 or 2 times daily; total dosage not to exceed 45 g weekly; not to be used with occlusive dressing
Moderate to severe systemic lupus erythematosus (SLE)
PREDNISONE
Adults: Up to 10 mg orally daily; may use alternate day dosing; severe disease 40-60 mg orally daily (maximum 100 mg orally daily)
METHOTREXATE SODIUM
Adults: 7.5-25 mg orally or IM once weekly
METHYLPREDNISOLONE
Adults: Up to 1 g IV infusion over 30 minutes once daily for 3 consecutive days
AZATHIOPRINE (Related toxicological information in AZATHIOPRINE/MERCAPTOPURINE)
Adults: 1-3 mg/kg orally daily
CYCLOPHOSPHAMIDE (Related toxicological information in CYCLOPHOSPHAMIDE AND RELATED AGENTS)
Adult: 0.5-1.0 g/m2 body surface area intravenously once monthly
Non-Procedural Therapy
Prevention of exacerbations and complications of systemic lupus erythematosus
* Lifestyle Modifications
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