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82 Cards in this Set
- Front
- Back
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Lupus nephritis class I
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Normal
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Lupus nephritis class II
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Mesangial proliferation
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Lupus nephritis class III
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Focal segmental glomerulonephritis
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Lupus nephritis class IV
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Diffuse proliferative glomerulonephritis
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Lupus nephritis class V
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Membranous nephritis
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Lupus nephritis class VI
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Glomerulosclerosis
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does myositis occur in SLE?
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suggests overlap syndrome such as mixed connective tissue disorder
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With which diseases are anti--Sm antibodies associated?
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SLE
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With which diseases are anti--RNP antibodies associated?
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SLE, mixed connective tissue disease
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With which diseases are anti-cardiolipin antibodies associated?
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anti-phospholipid antibody syndrome
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What risks are associated with Antiphospholipid antibody syndrome?
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arterial and venous thrombosis, recurrent fetal loss, Raynaud phenomenon, and thrombocytopenia. Neurologic involvement also may occur and may include chorea and transverse myelitis. Patients also are at risk for Libman-Sacks endocarditis
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Labs indicative of antiphospholipid antibody syndrome
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anticardiolipin antibodies, prolonged phospholipid-dependent coagulation tests (such as PTT, abnormal dilute Russell viper-venom time), and circulating lupus anticoagulant
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What is Neonatal Lupus Erythematosus?
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transplacental transfer of maternal antibodies result in hematologic, cutaneous, hepatic, and cardiac abnormalities
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What antibodies are most commonly involved in causin NLE?
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anti-Ro- or -La
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describe cutaneous lesions in NLE
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present after delivery or in first few weeks, occur on face, and resolve without scarring
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what is the most common heme findin in NLE?
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thrombocytopenia (usually no bleeding)
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When does NLE resolve?
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by 6 months
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What sequela of NLE is permanent?
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Congenital heart block is permanent because the fetal conducting system is damaged during its development; may be detected early in pregnancy as bradycardia in the fetus; requires placement of a permanent pacemaker. mortality still is significant
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What are the indications for hydroxychloroquine in SLE?
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. Hydroxychloroquine is used to reduce fatigue, mucocutaneous symptoms, and alopecia
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What medications are commonly used in SLE?
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Sunscreen, NSAIDS, Methotrexate, hydroxychloroquine, Glucocorticoids, IV IGCyclophosphamide for serious organ involvement; sometimes Azathioprine and cyclosporine
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What is indication for IV IG?
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chorea and thrombocytopenia.
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What are the causes of death in SLE?
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renal disease, infection, and CNS disease
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What are the most common presenting symptoms of SLE?
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fever, rash, mucositis and arthritis. malaise and weight loss.
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When are most pediatric patients diagnosed with SLE?
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adolescence
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female to male ratio
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3:1 prior to puberty 9:1 after puberty
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What ethnicity is most susceptible to SLE?
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Native Americans
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In what Ethnicities is SLE most severe?
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African americans and Hispanics
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What diseases are associated with a butterfly rash?
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SLE and dermatomyositi
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describe butterfly rash
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photosensitive, over cheeks and crosses nasal bride, spares nasolabial folds
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how does malar rash differ from rosacea?
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no pustules, papules nor telaniectasia
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What causes palmar erythema?
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vasculitic lesions on the palms
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Where are discoid lesions found?
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on the scalp or extremities
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Where are maculopapular lesions found?
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may occur anywhere
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describe the alopecia in lupus
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begins in front; rarely permanent
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describe the mucosal lesions in SLE?
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painless
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What are the most common CNS symptoms of SLE?
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psychiatric manifestations (including psychosis), seizures, an headaches
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less common CNS symptoms of SLE?
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chorea, neuropathies, and transverse myelitis
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Which layer of the heart is involved in SLE?
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Any layer, but most commonly the pericardium
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symptoms of pericarditis
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chest pain that is exacerbated when lying down or taking a deep breath; a friction rub may be heard on auscultation
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how does SLE myocarditis manifest?
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CHF or arrythmia
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Libman-Sacks endocarditis leads to ....
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sterile verrucous vegetations
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What % of people with SLE have Libman-Sacks endocarditis?
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up to 50%
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For what is a patient with Libman-Sacks endocarditis at risk?
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SBE
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Is Raynaud's phenomenon usually primary or secondary? How distinguish between the two?
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usually primary. Dilated or corkscrew shaped nailbed capillaries or dropout areas (a large gap between capillaries) suggests vasculopathy (secondary)
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How do you prophylax patients with frequent and prolonged Raynaud's?
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nifedipine (vasodilator) in the winter
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Why do SLE patients have a high risk of premature atherosclerosis?
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effects of glucocorticoids; nephrotic syndrome; antiphospholipid antibody
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What is the most common pulmonary manifestation of SLE in childhood?
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pleuritic chest pain due to effusion
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What % of adolescents with SLE have pulmonary disease?
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about 60%, much is subclinical
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What causes shrinking lung syndrome?
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a dysfunction in the diaphragm that elevates this organ, resulting in decreased lung volume
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GI symptoms in an SLE patient prompt suspicion of...
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pancreatitis (primary or secondary to steroids), mesenteric vasculitis, peritonitis, hepatitis
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What % of SLE pts have renal disease?
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~75% of children, often within the first 2 years of the disease
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musculoskeletal symptoms of SLE
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arthralgias or arthritis, myalgias, proximal muscle weakness
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describe SLE arthritis
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non erosive and non deforming
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what joints does SLE arthritis affect?
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both small and large joints
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what causes jaccoud arthropathy
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a nonerosive but deforming arthritis that results from joint subluxation
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common hematologic findings in SLE?
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cytopenias (all cell lines) are common at the onset; anemia occurs in ~ 50% of patients
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what kind of anemia is associated with SLE?
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anemia of chronic disease (normocytic normochromic) or autoimmune hemolytic anemia
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how is SLE diagnosed?
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clinical manifestations, autoantibodies
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Is ANA a good screening test for SLE?
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sensitive but not specific; positive in 33% of general healthy population
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What other diseases are associated with positive ANA?
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Juvenile Idiopathic Arthritis, Dermatomyositis, Scleroderma, Thyroid disease, recent infectious illness
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What auto-antibodies are more specific for SLE than ANA?
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anti-dsDNA; extractable nuclear antigens anti-Sm (Smith) and anti-RNP (ribonucleoprotein), anti-Ro (also called SS-A), anti-La (also called SS-B)
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I'M AN OPD RASH: I
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immune manifestations: anti-ds DNA or anti-Sm, false positive RPR, antiphospholipid antibodies (anticardiolipin or lupus anticoagulant)
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I'M AN OPD RASH: M
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malar rash
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I'M AN OPD RASH: A
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anti nuclear antibody elevation
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I'M AN OPD RASH: N
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neurologic manifestations: seizure or psychosis
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I'M AN OPD RASH: O
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oral ulcers
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I'M AN OPD RASH: P
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PHOTOSENSITIVITY
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I'M AN OPD RASH: D
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DISCOID RASH
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I'M AN OPD RASH: R
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RENAL DISEASE
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I'M AN OPD RASH: A
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ARTHRITIS
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I'M AN OPD RASH: S
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SEROSITIS: PLEURITIS OR PERICARDITIS
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I'M AN OPD RASH: H
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HEMATOLOGIC: ANEMIA, LEUKOPENIA, LYMPHOPENIA, THROMBOCYTOPENIA
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Which antibody is most known for its association with neonatal lupus erythematosis (NLE) and the development of congenital heart block?
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anti-Ro antibodies
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serologic markers of active SLE?
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reduced levels of C3 or C4, elevated anti-ds DNA, ESR and CRP
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% of anti-ds DNA in SLE
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73%
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% SLE positive for anti-Ro (anti-SSA)
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40%
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% anti-SSB (anti-La) in SLE
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10-15%
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% of anti-Sm antibodies in SLE
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20-30%
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% of anti-cardiolipin in SLE
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37%
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% anti-RNP antibodies in SLE
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15%
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anti-Ro associated with....
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SLE (especially with cutaneous manifestations), Sjogren syndrome, NLE
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Which diseases are associated with anti-La?
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SLE, sjogren, NLE
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