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RH12 Rheumatology
Connective Tissue Disorders Toronto Notes 2019 Table 15. Signs and Symptoms of SLE
Raynaud’s Phenomenon
Vasospastic disorder characteristically causing discolouration of fingers and toes (white → blue → red)
Classic triggers: cold and emotional stress
System Systemic Renal Dermatologic
Musculoskeletal Ophthalmic
Cardiac Vascular
Respiratory Gastrointestinal Neurologic/Psychiatric Life/Organ-Threatening
Investigations
Symptoms
Fatigue, malaise, weight loss, fever, lymphadenopathy
Hematuria, proteinuria (glomerulonephritis), HTN, peripheral edema, renal failure
Photosensitivity, malar rash, discoid rash, oral ulcers, alopecia (hair loss), purpura, panniculitis (inflammation of subcutaneous fat and muscle tissue), urticaria
Polyarthralgias, polyarthritis, myalgias, AVN; reducible deformities of hand = Jaccoud’s arthritis
Keratoconjunctivitis sicca, episcleritis, scleritis, cytoid bodies (cotton wool exudates on fundoscopy = infarction of nerve cell layer of retina)
Pericarditis, CAD, non-bacterial endocarditis (Libman-Sachs), myocarditis Note: SLE is an independent risk factor for atherosclerosis and CAD
Raynaud’s phenomenon, livedo reticularis (mottled discolouration of skin due to narrowing of blood vessels, characteristic lacy or net-like appearance), thrombosis, vasculitis
Pleuritis, ILD, pulmonary HTN, PE, alveolar hemorrhage
Pancreatitis, SLE enteropathy, hepatitis, hepatomegaly, splenomegaly
H/A, depression, psychosis, seizures, cerebritis, transverse myelitis, peripheral neuropathy, stroke
Cardiac: coronary vasculitis, malignant HTN, tamponade
Hematologic: hemolytic anemia, neutropenia, thrombocytopenia, TTP, thrombosis Neurologic: seizures, CVA, stroke
Respiratory: pulmonary hypertension, pulmonary hemorrhage, emboli
Consider SLE in a patient who has involvement of 2 or more organ systems
• ANA(98%sensitivity,butpoorspecificity→usedasascreeningtest;ANAtitresarenotusefultofollow disease course)
• anti-dsDNAandanti-Smarespecific(95-99%)
• anti-dsDNAtitreandserumcomplement(C3,C4)areusefultomonitortreatmentresponseinpatients
who are clinically and serologically concordant (anti-dsDNA, C3, and C4 also fluctuate with disease
acitivity)
• antiphospholipidAb(anti-cardiolipinAb,SLEanticoagulant,anti-β2glycoprotein-IAb),maycause
increased risk of clotting and increased aPTT
Treatment
• goalsoftherapy
■ usually immunosuppressive ± corticosteroid
■ treat early and avoid long-term steroid use, if unavoidable see Endocrinology, E41 for osteoporosis
management
■ if high doses of steroids necessary for long-term control, add steroid-sparing agents and taper when
possible
■ treatment is tailored to organ system involved and severity of disease
■ all medications used to treat SLE require periodic monitoring for potential toxicity
• dermatologic
■ sunscreen, avoid UV light and estrogens ■ topical steroids, hydroxychloroquine
• musculoskeletal
■ NSAIDs ± gastroprotective agent for arthritis (also beneficial for pleuritis and pericarditis) ■ hydroxychloroquine improves long-term control and prevents flares
■ bisphosphonates, calcium, vitamin D to combat osteoporosis
• organ-threateningdisease
■ high-dose oral prednisone or IV methylprednisolone in severe disease
■ steroid-sparing agents: azathioprine, MTX, mycophenolate (can use mofetil or sodium)
■ IV cyclophosphamide for serious organ involvement (e.g. cerebritis or lupus nephritis) for clinical
features of lupus nephritis
Antiphospholipid Antibody Syndrome
Definition
• multi-systemvasculopathymanifestedbyrecurrentthromboembolicevents,spontaneousabortions, and thrombocytopenia
• circulatingantiphospholipidautoantibodiesinterferewithcoagulation
• primaryAPLS:occursintheabsenceofotherdisease
• secondaryAPLS:occursinthesettingofaconnectivetissuedisease(includingSLE),malignancy,drugs
(hydralazine, procainamide, phenytoin, interferon, quinidine), and infections (HIV, TB, hepatitis C,
infectious mononucleosis)
• catastrophicAPLS:developmentwithin1wkofsmallvesselthromboticocclusionin≥3organsystems
The arthritis of SLE can be deforming but it is non-erosive (in contrast to RA)
with positive antiphospholipid Ab (high mortality)