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Where are mucosal ulcerations associated with SLE typically found?
Approximately 10% of patients with SLE develop mucosal ulcerations that characteristically are localized to the tongue and hard palate but may affect the nose and entire mouth. They typically are painless but may cause pain in some patients.
What is discoid lupus?
- form of chronic cutaneous lupus and occurs in approximately 5% of patients with SLE. This condition can exist as a primary cutaneous disease as well as a manifestation of SLE; only 10% of patients with primary discoid lupus develop SLE.
- Discoid lupus manifests as erythematous indurated scaly plaques that typically develop above the neck and often involve the ear canals. Key features include peripheral expansion and central regression with plugging of the hair follicles, hyperpigmentation, and atrophic scarring. Facial scarring can be severe, and scarring on the scalp can cause irreversible patchy alopecia.
What does the joint involvement in SLE look like?
What is Jaccoud arthropathy?
More than 90% of patients with SLE develop joint involvement that can manifest as arthralgia or true arthritis. Joint pain is often migratory and can be oligoarticular or polyarticular, or asymmetric or symmetric. Pain typically involves the large and small joints; the wrists and metacarpophalangeal and proximal interphalangeal joints in particular are most commonly affected.Tenderness, swelling, and warmth of the joints also can occur in patients with SLE and are often mild, and arthritis in this setting is typically nonerosive. Tendon inflammation can cause joint laxity that leads to reducible deformities that mimic rheumatoid arthritis, such as Jaccoud arthropathy. Patients with SLE also may develop fibromyalgia.
What are signs of Proliferative Lupus Nephritis?
What would the renal biopsy show?
- Signs of proliferative lupus nephritis may include new-onset hypertension or edema. Laboratory studies in affected patients typically reveal high titers of anti–double-stranded DNA antibodies and hypocomplementemia, proteinuria, hematuria, and erythrocyte and granular casts in the urine.
- Renal biopsy specimens in patients with these conditions characteristically reveal glomerular hypercellularity, crescent formation, and immune complex deposition in the subendothelial space and may show interstitial inflammation.
How do you treat Proliferative Lupus Nephritis?
Patients with early disease may not have renal insufficiency, and early treatment does not significantly affect the findings on renal biopsy. However, progression to renal failure can be rapid; therefore, patients with a strong clinical suspicion for lupus nephritis should begin high-dose corticosteroid therapy before renal biopsy is performed.
What are the features of Membranous Lupus Nephritis? How do you differentiate this from Proliferative glomerulonephritis?
Patients with membranous lupus nephritis typically present with the nephrotic syndrome and usually do not have features of nephritis, such as hypertension, an active urine sediment, or renal insufficiency. High titers of anti–double-stranded DNA antibodies and hypocomplementemia also may be absent.
What is the pathological characteristic of membranous lupus nephritis?
Membranous lupus nephritis is characterized pathologically by thickening of the basement membrane, immune complex deposition in the subepithelial area, and an absence of hypercellularity in the glomerulus.
What are the manifestations of Neurologic involvement in SLE?
Antineuronal antibodies may be present, but elevated titers of anti–double-stranded DNA antibodies and hypocomplementemia often are absent. Manifestations include peripheral neuropathy, mononeuritis multiplex, cranial neuritis, transverse myelitis, aseptic meningitis, stroke, seizure, encephalitis, and psychosis.
What is an adverse effect of NSAIDs in patients with SLE?
What is acute lupus pneumonitis?
Acute lupus pneumonitis is a rare but serious condition that generally manifests as fever, cough, pleurisy, and shortness of breath accompanied by infiltrates on chest radiography. Differentiating between acute lupus pneumonitis and infection is critical; occasionally, lung biopsy is needed for diagnosis.
What suggests alveolar hemorrhage in a pt with SLE?
Alveolar hemorrhage as a manifestation of pulmonary vasculitis is suggested by alveolar infiltrates on chest radiography and an increased DLCO
Describe the joint involvement in SLE.
- Joint pain is often migratory and can be oligoarticular or polyarticular, or asymmetric or symmetric. Pain typically involves the large and small joints; the wrists and metacarpophalangeal and proximal interphalangeal joints in particular are most commonly affected.
- Tenderness, swelling, and warmth of the joints also can occur in patients with SLE and are often mild, and arthritis in this setting is typically nonerosive. Tendon inflammation can cause joint laxity that leads to reducible deformities that mimic rheumatoid arthritis, such as Jaccoud arthropathy. Patients with SLE also may develop fibromyalgia.
What kind of hematologic involvement occur in SLE?
- Coomb's positive hemolytic anemia
- Leukopenia (as a result of lymphopenia, not neutropenia). This is a good marker of disease activity.
A pt with SLE presents with neurologic or renal manifestations. What is the next intervention?
- Approximately 40% of patients with SLE have antiphospholipid antibodies. However, not all of these patients have manifestations of the antiphospholipid syndrome, which include venous and arterial thrombosis and recurrent fetal loss. This syndrome also may be associated with thrombocytopenia, hemolytic anemia, livedo reticularis, and cardiac valvular disease.
- Both SLE and the antiphospholipid syndrome can cause neurologic and renal manifestations. Therefore, determining whether the presenting condition in patients with SLE who have antiphospholipid antibodies is inflammatory or thrombotic/embolic is critical in order to initiate appropriate treatment. The presence of inflammatory markers such as an elevated erythrocyte sedimentation rate, leukopenia, elevated titers of anti–double-stranded DNA antibodies, and hypocomplementemia suggest an inflammatory process, whereas tissue infarcts in the absence of elevated inflammatory markers and thrombotic vasculopathy on tissue biopsy specimen suggest a thrombotic process. Anticoagulation is indicated in patients with thrombotic manifestations of the antiphospholipid syndrome.
What lab tests are indicated in the workup of a pt with clinical findings diagnostic of SLE?
- Further testing indicated in patients with symptoms suggestive of SLE include a complete blood count, erythrocyte sedimentation rate measurement, and urinalysis. Patients with a high pretest probability of SLE and antinuclear antibodies (usually a titer ≥1:160) should undergo...
- Confirmatory testing, such as measurement of C3, C4, and CH50 and more specific autoantibody testing. Antibodies to double-stranded DNA are present in approximately 50% to 70% of patients with SLE, whereas anti-Smith, antiribonucleoprotein, anti-Ro/SSA, and anti-La/SSB antibodies are present in 10% to 60% of patients with SLE. These antibodies are rarely present in patients without connective tissue disease. Patients with a new diagnosis of SLE also should also undergo screening for anticardiolipin antibodies and the lupus anticoagulant.
What medications have definite associations with Drug-Induced Lupus?
"HIP Me Call Quin Miry"
Symptoms of Drug-induced lupus typically lasts how long?
Symptoms typically lasts 4-6 weeks after withdrawal of the offending drug. Some affected patients may require a short course of NSAIDs or corticosteroids.
What is Undifferentiated Connective Tissue Disease?
Undifferentiated connective tissue disease (UCTD) is characterized by the presence of antinuclear antibodies accompanied by only one or two criteria for SLE. Most patients with UCTD do not have progressive disease, although some affected patients may eventually develop SLE or another connective tissue disease, such as systemic sclerosis or myositis. Treatment of UCTD involves symptomatic management.
What lab work picture do you typically see on a pt with drug-induced lupus?
Patients with drug-induced lupus typically have anti-nuclear and antihistone antibodies but do not have renal or neurologic involvement or antibodies to anti-double-stranded DNA, anti-Smith, anti-Rnp, or anti-Ro/SSA or anti-La/SSB
What are the treatment options for SLE?
- 1. Immunizations and PPD
- 2. NSAIDs and Hydroxychloroquine
- 3. Corticosteroids
- 4. Immunosuppressive agents
- Patients with SLE should receive all routinely prescribed immunizations; however, patients taking more than 20 mg/d of prednisone or who use immunosuppressive therapy should not receive live attenuated vaccines, including those for varicella, herpes zoster virus, mumps, measles, and rubella. Tuberculin skin testing using purified protein derivative also should be performed in patients with SLE in whom corticosteroid or immunosuppressive therapy will most likely be used.
- NSAIDs can be used to relieve pain
- COX-2 may increase the risk for thrombosis and therefore should be used with caution in patients with SLE who have antiphospholipid antibodies.
- Hydroxychloroquine is well tolerated and has a good safety profile and significant disease-modifying properties. This agent is effective for skin and joint manifestations and helps to prevent disease flares. Hydroxychloroquine should be continued indefinitely to prevent disease reactivation, even if the disease has been quiescent for many years.
- Cyclophosphamide, mycophenolate mofetil, and azathioprine have a steroid-sparing effect and have been shown to improve outcomes in patients with severe SLE, particularly those with renal involvement. In patients with lupus nephritis, combination therapy with monthly intravenous cyclophosphamide and high-dose corticosteroids is superior to corticosteroids alone.
- Cyclophosphamide is associated with serious toxicity; therefore, after remission of renal disease has been achieved and maintained for 3 to 6 months, switching from cyclophosphamide to mycophenolate mofetil or azathioprine should be considered. Mycophenolate mofetil is as effective as cyclophosphamide in inducing remission of lupus nephritis, but long-term outcomes of this therapy remain uncertain.
What is an optimal time of disease regression before an SLE pt should get pregnant?
- Patients whose disease has been quiescent for at least 6 months before conception and who take either no medications or medications that can be continued during pregnancy typically have favorable pregnancy outcomes.
- The risk of disease flare during pregnancy is increased sevenfold if the disease has been active 6 months before pregnancy or if hydroxychloroquine has been discontinued.
What SLE medications should be continued during pregnancy if they are controlling the disease?
Patients whose disease is controlled by corticosteroids, hydroxychloroquine, or azathioprine should continue therapy during pregnancy.
What medications should be not be used during pregnancy of an SLE pt?
Cyclophosphamide, mycophenolate mofetil, methotrexate, warfarin, angiotensin-converting enzyme inhibitors, and bisphosphonates should not be used during pregnancy.
If a pt with SLE and history of antiphospholipid antibodies (or a history of recurrent fetal loss) now present with her 1st pregnancy, what interventions need to be done?
Antiphospholipid antibodies predispose patients to intrapartum and postpartum venous thromboembolism. These antibodies also are associated with second- and third-trimester pregnancy losses caused by placental thrombosis and placental insufficiency. Subcutaneous heparin should be instituted in patients with antiphospholipid antibody positivity and a history of recurrent fetal loss or thrombosis. Ideally, heparin should be initiated when patients begin trying to conceive. Low-dose aspirin is often used in conjunction with heparin.
What intervention is needed for pregnant pts with a history of anti-Ro/SSA or anti-La/SSB?
- Neonatal lupus affects 1% to 2% of children of mothers with anti-Ro/SSA or anti-La/SSB antibodies, independent of whether these women have SLE or Sjögren syndrome.
- Pregnant patients who have anti-Ro/SSA or anti-La/SSB antibodies should undergo regular fetal echocardiography starting at 16 weeks of gestation.
What are routine follow-up labs and procedures for pts with SLE?
- Patients with SLE should be asked about symptoms during each office visit.
- A complete blood count, measurement of anti–double-stranded DNA antibodies and C3, and urinalysis also are indicated to help assess disease activity.
- Management of SLE also involves aggressive treatment of hypertension, hyperlipidemia, and hyperglycemia; weight control; smoking cessation; and age- and sex-appropriate malignancy screening.