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MSK II Exam 2 > Kinder CIS- Lupus > Flashcards

Kinder CIS- Lupus Flashcards

1
Q

Criterion for SLE

A

4 of the 11:

Malar rash
Discoid rash
Photosensitivity
Oral ulcers-
Nonerosive arthritis
pleuritis or pericaritis
Renal Disorder
Neurologic Disorder
Hematologic Disorder
Immunologic Disorder
Positive Antinuclear Antibody
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2
Q

Malar rash of SLE

A

Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds

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3
Q

Discoid rash of SLE

A
  • erythrematous raised patches with adherent keratotic scaling and follicular plugging, atrophic scarring may occur in older lesions
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4
Q

Photosensitivity of SLE

A
  • skin rash as a result of unusual reation to sunlight, by patient history or physician observation
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5
Q

Oral ulcers of SLE

A

oral or nasopharyngeal ulceration, usually painless, observed by physician

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6
Q

Nonerosive arthritis of SLE

A
  • involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion
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7
Q

pleuritis or pericaritis of SLE

A
    1. pleuritis- convincing history or pleuritic pain or rubbing heard by a physican of evidence of pleural effusion - OR-
      pericarditis- documented by electrocardiogram or rub or evidence of pericardial effusion
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8
Q

Renal Disorder of SLE

A

Persistent proteinuria > 0.5 grams per day or > than 3+ if quantitation not performed –OR—
Cellular casts – may be red cell, hemoglobin, granular, tubular, or mixed

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9
Q

Neurologic Disorder of SLE

A

Seizures – in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance –OR—
Psychosis – in the absence of offending drugs or know metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance

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10
Q

Hematologic Disorder of SLE

A

Hemolytic anemia – with reticulocytosis –OR—
Leukopenia – less than 4,000/mm3 on ≥ 2 occasions –OR—
Lymphopenia – less than 1,500/mm3 on ≥ 2 occasions –OR—
Thrombocytopenia – less than 100,000/mm3 in the absence of offending drugs

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11
Q

Immunologic Disorder of SLE

A

Anti-DNA: antibody to native DNA in abnormal titer –OR—
Anti-Sm: presence of antibody to Sm nuclear antigen –OR—
Positive finding of antiphospholipid antibodies

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12
Q

Positive Antinuclear Antibody

of SLE

A

An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs

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13
Q

Systemic Lupus Erythematosus etiology/ characteristics

A

Female predominance 10-15:1
Unknown etiology
- Genetic, hormonal, and environmental influences play a role in the disease
- Smoking is a risk factor for SLE and UV light can trigger exacerbations

SLE is characterized by the production of autoantibodies to:

  • Nuclear antigens - ANA
  • Cytoplasmic antigens
  • Cell surface antigens
  • Soluble antigens – IgG and phospholipids
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14
Q

Lupus autoantibodies and timeline

A 
First to appear 3.4 years prior to diagnosis
ANA
anti-Ro/SSA
anti-La/SSB
Antiphospholipid

Second to appear 2.2 years prior to diagnosis
anti-dsDNA

Third to appear 1 year prior to diagnosis
anti-Sm
anti-RNP

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15
Q

constitutional and mucocutaneous symptoms of lupus

A

Constitutional
Fever, fatigue, and weight loss

Mucocutaneous 80-90% of patients
Alopecia
Nasal and oral ulcers – painless
Acute cutaneous lupus erythematosus (ACLE)
Subacute cutaneous lupus erythematosus (SCLE)
Chronic cutaneous lupus erythematosus (CCLE)

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16
Q

Acute Cutaneous Lupus Erythematosus (ACLE)

A

Localized
- Classic malar rash
— Sparing of nasolabial folds
— Differential diagnosis: acne rosacea, seborrheic dermatitis, and flushing syndromes
Rosacea – telangiectasias, pustules, heat and alcohol make it worse
Seborrehic dermatitis – scaly, erythematous plaques on the eyebrows and lateral sides of nose including the nasolabial folds

Generalized

  • Maculopapular erythematosus lesions involving any area of the body in a photosensitive distribution
  • Dorsa of hands and the extensor surfaces of the fingers are commonly involved
  • Erythema is typically found between the interphalangeal joints and spares the knuckles, unlike Gottron papules
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17
Q

Subacute Cutaneous Lupus Erythematosus

A 
Papulosquamous or annular
Non scarring
Most photosensitive
Torso, Limbs, and spares face
Associated with anti-SSA/Ro antibody
Can be induced by medicines
- Hydrochlorothiazide
- terbinafine
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18
Q

Chronic Cutaneous Lupus Erythematosus

A

Discoid Lupus

  • Raised, erythematous plaques with adherent scale
  • Scalp, face, and neck
  • Erythematous ring around lesion – active component
  • Can lead to scarring, skin atrophy, permanent alopecia, and disfigurement

Lupus panniculitis
- Deep, firm nodule that can lead to cutaneous atrophy and rarely ulceration

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19
Q

Musculoskeletal stuff in SLE

A

Arthritis and arthralgias in up to 95% of SLE patients
Commonly involve wrists, and small joints of hands
Boney erosions rarely occur
Swan neck deformity and ligamental laxity often noted
- Usually reducible

20
Q

Lupus Nephritis

A

Significant cause of morbidity and mortality
90% of SLE patients have pathologic evidence of nephritis on biopsy
Clinically significant lupus nephritis occurs in 50% of patients with SLE
Usually develops in the first 3 years of the disease
Immune complex glomerulonephritis is most common, but SLE patients may have tubulointerstitial disease or vascular disease

Clinical presentation – highly variable

  • Asymptomatic hematuria
  • Nephrotic Syndrome
  • Rapidly progressive glomerulonephritis with loss of renal function
21
Q

Lupus nephritis screening

A

Screening
History
- Polyuria, nocturia, or foamy urine

Examination
- Blood pressure and Lower extremity edema

Urinalysis

  • Hematuria, pyuria, dysmorphic blood cells, and red cell casts
  • Proteinuria
  • Screen patients every 3 months with active disease

Biopsy

  • Patients with clinical or laboratory findings suggestive of nephritis should have a renal biopsy
    • Confirms Diagnosis
    • Evaluates disease activity
    • Determines appropriate treatment
22
Q

Classes of Lupus Nephritis

A

Class I and Class II nephritis
Excellent renal prognosis
Does not require specific treatment

Class III and Class IV nephritis
Long-term prognosis is extremely poor without immunosuppression

Class V
Better prognosis than class III or IV
Proliferative changes portend a worse prognosis
23
Q

Cardiovascular stuff with Lupus

A

May involve pericardium, valves, myocardium, and coronary arteries
Raynaud’s affects 30% of SLE patients
- Vasospasm of the digital arteries and arterioles after exposure to cold or stress

Pericarditis

  • May be asymptomatic with small pericardial effusions
  • On occasion can be life threatening from hemodynamic complications

Valves

  • Mitral and Aortic Valve leaflets with valve thickening
  • Libman-Sacks endocarditis – nonbacterial vegetations

Myocarditis and conduction defects are rare

SLE is associated with accelerated atherosclerosis and is a risk factor for coronary artery disease

24
Q

Pulmonary stuff with SLE

A

Pleural effusions, typically small develop in 50% of patients

Pleuritic chest pain

Lupus pneumonitis

  • Extremely rare
  • Acute respiratory illness with fever, cough, and pulmonary infiltrates
  • High Mortality

Chronic interstitial lung Disease
- Rare

Diffuse Alveolar Hemorrhage

  • Rare and frequently fatal
  • Acute pulmonary infiltrates, falling hematocrit, and hemorrhagic bronchoalveolar lavage
  • Lupus nephritis frequently associated
25
Q

Gastrointestinal issues in SLE

A

Abdominal pain occurs in 40% of SLE patients

  • Important to rule out non SLE etiologies first in immunocompromised patients. Immunosuppressives may mask clinical signs of acute infection
    Peritonitis, pancreatitis (rare), mesenteric vasculitis, and intestinal pseudo-obstruction
    Lupus Hepatitis
    Vascular Disorders of the Liver (antiphospholipid antibodies)
  • Budd-Chiari, hepatic veno-occlusive disease, and hepatic infarction
26
Q

Neuropsychiatric issues in lupus

A

Headaches
Cerebrovascular Disease
Seizures
Mood Changes
Cognitive Dysfunction
MRI of brain may show T2 hyperintense focal lesions in the periventricular and subcortical white matter that look like multiple sclerosis lesions
Lumbar puncture should be performed in CNS disease to rule out infection

27
Q

a more rare neurologic condition in lupus

A

Acute Myelopathy (Transverse Myelitis)
Uncommon, but devastating
Bilateral lower extremity paresthesia, numbness, and weakness that can progress rapidly to the upper limbs and muscles of respiration
Sensory level usually present
MRI to confirm diagnosis
- T1 and T2 signal abnormalities and widening of the cord from edema are observed
Urgent treatment necessary to prevent permanent damage

28
Q

strokes and neuropathies in lupus

A

Stroke
- Thrombotic secondary to antiphospholipid antibodies

Peripheral Neuropathy
- 20% of lupus patients

Large Fiber Vasculitic neuropathy
- Requires urgent treatment to prevent permanent nerve damage

Autonomic neuropathies

Cranial Nerve neuropathies

29
Q

Anemias in lupus

A 
Anemia of chronic disease – most common
Autoimmune hemolytic anemia
Microangiopathic hemolytic anemia
Blood Loss
Renal insufficiency
Pure red cell aplasia
Aplastic anemia
30
Q

Autoimmune Hemolytic Anemia

A 
Increased serum unconjugated bilirubin
Increased LDH
Increased reticulocyte count
Reduced serum haptoglobin
Direct Coombs typically positive
Peripheral smear with spherocytosis
Association with anticardiolipin antibodies
May predate lupus by many years
31
Q

Microangiopathic Hemolytic Anemia

A

Peripheral smear with schistocytes

Consider thrombotic thrombocytopenic purpura

  • MAHA, thrombocytopenia, fever, neurologic symptoms, and renal involvement
  • Check antiphospholipid antibodies
  • – MAHA, thrombocytopenia, neurologic symptoms, and renal involvement can occur with catastrophic antiphospholipid antibody syndrome
32
Q

Acute Phase Reactants in lupus

A

Erythrocyte Sedimentation Rate (ESR)

  • Correlates with disease activity
  • Nonspecific

C-reactive protein

  • Does not correlate well with active disease
  • Can be useful in patients with serositis and concomitant infection in which the CRP level can be very elevated
33
Q

Anti-dsDNA

A

95% specificity for SLE; fluctuates with disease activity; associated with glomerulonephritis

34
Q

Anti-Sm

A

99% specificity for SLE; associated with anti-U1RNP antibodies

35
Q

Anti-U1RNP

A

Defining antibody in MCTD; associated with lower frequency of glomerulonephritis

36
Q

Anti-Ro/SSA

A

Associated with Sjögren syndrome, photosensitivity, SCLE, neonatal lupus, congenital heart block

37
Q

Anti-La/SSB

A

Associated with Sjögren syndrome, SCLE, neonatal lupus, congenital heart block, anti-Ro/SSA

38
Q

Anti-histone

A

Associated with drug-induced lupus

39
Q

Antiphospholipid

A

Associated with arterial and venous thrombosis, pregnancy morbidity

40
Q

Complement

A

Hypocomplementemia occurs in SLE flares

C4, C3, and CH50

41
Q

lupus Differential Diagnosis

A

Parvovirus B19
- Fever, rash, anemia, and symmetric inflammatory polyarthritis
ANA, anti-dsDNA antibodies, and hypocomplementemia

Cytomegalovirus and EBV
- Constitutional symptoms; cytopenias; GI, hepatic, and lung abnormalities

Acute HIV Infection
- Fever, lymphadenopathy, and mucosal ulcers
Hepatitis B and C
Inflammatory arthritis with positive autoantibodies

Non-Hodgkin lymphoma

  • Constitutional symptoms
  • Arthralgias
  • Cytopenias
  • Rash
  • Positive ANA

Autoimmune Diseases

  • Rheumatoid arthritis
    • anti-CCP usually absent in SLE
  • Dermatomyositis
  • MCTD
  • – usually high titer anti-RNP antibody
  • – arthritis is usually erosive
  • – Pulmonary Hypertension leading cause of morbidity and mortality

Drug Induced Lupus

  • Polyarthritis, myalgia, fever, and serositis
  • Minocycline, procainamide, hydralazine, isoniazid, interferon alpha, anti-TNF drugs
  • HCTZ associated with SCLE
  • Anti-Histone antibodies
42
Q

Complications of lupus

A

Accelerated Atherosclerosis
- Myocardial Infarction in women with SLE is 50 times more common than in age-matched controls

End-Stage Renal Disease

  • 10% of lupus nephritis patients progress to end-stage renal disease requring dialysis
  • SLE patients are good candidates for renal transplant

Infections

  • Bacterial, viral, and opportunistic
  • Progressive multifocal leukoencephalopathy
  • – Rare and usually fatal secondary to reactivation of th JC polyomavirus

Osteoporosis and Avascular Necrosis
- Secondary to glucocorticoids

Malignancy

  • Non-Hodgkin lymphoma
  • Hodgkin lymphoma
  • Lung cancer
  • Cervical cancer
43
Q

Prognosis for lupus

A

1950s
50% survival at 2 years

Current
90% survival at 10 years

Bimodal mortality

  • Early years secondary to active disease or infection
  • Long-standing disease with death secondary to atherosclerosis and malignancy
44
Q

Treatment of lupus

A

Glucocorticoids – cutaneous, musculoskeletal, serositis, neuropsychiatric, hemolytic anemia, alveolar hemorrhage
NSAID – musculoskeletal, serositis
Colchicine - serositis
Hydroxychloroquine – cutaneous, musculoskeletal
Methotrexate – musculoskeletal steroid sparing agent
Azathioprine – lupus nephritis
Mycophenolate mofetil – lupus nephritis
Cyclophospamide – lupus nephritis

45
Q

Lupus and Pregnancy

A 
Increased risk of disease flares during pregnancy
Thrombosis risk increased
Pregnancy should be delayed until disease well controlled for 6 months
Pregnancy outcome affected by
- Disease status at time of conception
- Renal Disease
- Antiphospholipid antibodies
- Anti-Ro/SSA and anti-La/SSB antibodies

Proteinuria, antiphospholipid syndrome, thrombocytopenia, and hypertension
- 30-40% Risk of pregnancy loss

46
Q

Neonatal Lupus

A

Affects children born of mothers with anti-SSA/Ro or anti-SSB/La antibodies

Transient

  • Rash – Subacute Cutaneous Lupus Erythematosus
  • Thrombocytopenia
  • Hemolytic Anemia
  • Arthritis

Permanent
- Complete Heart Block

Treatment
- Hydroxychloroquine

MSK II Exam 2 (16 decks)

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