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MSK II Exam 2 > Kinder CIS- Antiphospholipid Antibody Syndrome > Flashcards

Kinder CIS- Antiphospholipid Antibody Syndrome Flashcards

1
Q

Antiphospholipid Antibody Syndrome

A

Acquired disorder associated with circulating autoantibodies to anionic phospholipids and their protein binding complexes

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2
Q

Antiphospholipid Antibody Syndrome Clinical manifestations

A

Thromboses – can affect arterial and venous circulation
Pregnancy loss

Can be Primary or Secondary Antiphospholipid Syndrome

Secondary:
Systemic Lupus Erythematosus
Malignancy
Infections (HIV)
Drugs: phenytoin, chlorpromazine
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3
Q

Antiphospholipid Antibody Syndrome Deep venous thrombosis

A 
Lower extremities
Pulmonary Embolism
Renal vein thrombosis
Retinal vein occlusion
Budd-Chiari syndrome
Adrenal hemorrhage secondary to thrombosis of the adrenal veins
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4
Q

Antiphospholipid Antibody Syndrome Arterial Disease

A

in the skin- Ulceration, gangrene, livedo reticularis

cerebrovascular: Stroke, transient ischemic attack, seizure, encephalopathy, chorea, myelopathy, mononeuritis

Cardiac: Myocardial infarction, cardiomyopathy, valvular vegetations and abnormalities

Renal: Renal infarction, thrombotic microangiopathy

Pulmonary: Pulmonary emboli, microvascular thromboses, pulmonary hypertension, alveolar hemorrhage

Gastrointestinal: Mesenteric ischemia, splenic infarction

Ophthalmologic: Retinal artery thrombosis and ischemia

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5
Q

Antiphospholipid Antibody Syndrome Pregnancy morbidity

A

Fetal demise can occur at all gestational ages
Spontaneous abortions that occur from second trimester on are more specific for APS
Linked to preeclampsia
Linked to intrauterine growth retardation
HELLP syndrome (hemolysis, elevated liver enzymes, low platelets)

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6
Q

Antiphospholipid Antibody Syndrome clinical and lab criteria

A

Clinical Criteria:
Vascular Thrombosis
One or more clinical episodes without evidence of inflammation in the vessel wall

Pregnancy morbidity

  • One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation
  • One or more premature births of a morphologically normal neonate before the 34th week of gestation because of:
  • – Eclampsia or severe preeclampsia, or
  • – Recognized features of placenta insufficiency
  • Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation

Laboratory Criteria

  • Lupus anticoagulant in plasma
  • Anticardioplipin antibody (IgG or IgM) present in medium or high titer
  • Anti-β₂-glycoprotein I antibody (IgG or IgM) present in titer above the 99th percentile
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7
Q

Antiphospholipid Antibody Syndrome Diagnosis

A

Patients should satisfy one or more clinical criteria and one or more laboratory criteria
Laboratory Criteria should be present on two or more occasions at least 12 weeks apart

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8
Q

Antiphospholipid Antibody Syndrome- the antiphospholipid antibodies

A

Antiphospholipid Antibodies
5-10% of healthy population
50% of patients with SLE

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9
Q

Antiphospholipid Antibody Syndrome Strongest thrombosis risk

A

Lupus anticoagulant, followed by Anti β₂-glycoprotein, followed by anticardiolipin antibodies
Highest risk of thrombosis occurs when all three antibody types present

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10
Q

Antiphospholipid Antibody Syndrome Anticardiolipin Antibodies

A

Cross reacts with Rapid Plasma Reagin (RPR)
- False positive for syphilis
Greater sensitivity, but less specific than antibodies to β₂-glycoprotein I or lupus anticoagulants
Acute or chronic infections, especially viral, can induce anticardiolipin antibodies that do not correlate with APS and do not confer a risk of thrombosis

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11
Q

Antiphospholipid Antibody Syndrome Anti-β₂-glycoprotein I Antibodies

A

More specific than anticardiolipin antibodies

Not seen in the context of viral infections

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12
Q

Antiphospholipid Antibody Syndrome Lupus Anticoagulant activity

A

Antibodies that prolong in vitro phospholipid-dependent clotting assays, such as the Russel viper venom time
Not specific for SLE
Associated with risk of thrombosis, not bleeding
Antibody presence is inferred from their ability to interfere with a functional clotting assay in vitro
Patients with APS may have lupus anticoagulant activity but lack detectable anticardiolipin and anti-β2-glycoprotein I antibodies.

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13
Q

Antiphospholipid Antibody Syndrome coagulation tests

A

Prolongation of in vitro coagulation test in which phospholipids are added to enhance the clotting reaction – dilute Russell viper venom time (dRVVT) or PTT

Antiphospholipid antibodies interfere with the added phospholipid causing prolongation of the clotting time

1:1 mix of patient and control plasma must fail to completely correct the abnormal clotting time, indicating presence of an inhibitor, rather than a factor deficiency is responsible for the abnormality

Inhibitor must be shown to be phospholipid-dependent by demonstrating normalization of the clotting time by the addition of molar-excess concentrations of the phospholipid reagent

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14
Q

flow chart where LAC is positive

A 
Step 1: functional clotting assay.  Prolonged clotting time?  Yes -->
Mixing study (1:1 patient/ control) -->  if clotting time corrects, it's a factor deficiency.  if NOT-->

Addition of excess phospholipid. Corrects? POSITIVE for LAC

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15
Q

Antiphospholipid Antibody Syndrome treatment

A

Heparin
Warfarin

Pregnant Women
Heparin and ASA

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16
Q

Scleroderma Most frequent symptoms

A 
Raynaud phenomenon
Gastroesophageal reflux with or without dysmotility
Skin changes
Swollen fingers
Arthralgias

4-5 times more common in women

Average age of diagnosis is 50 years

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17
Q

Scleroderma Diagnosis

A

A) Thickened skin changes proximal to the metacarpophalangeal joints

OR

B) Two out of the three following

1) Sclerodactyly
2) Digital pitting (loss of tissue on the finger pads due to ischemia
3) Bibasilar pulmonary fibrosis

18
Q

Limited Scleroderma

A

Three or more features of the CREST Syndrome

Calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia

19
Q

Early or mild expression of Scleroderma

A

Raynaud’s phenomenon

with abnormal capillary loops or autoantibodies associated with scleroderma

20
Q

Scleroderma Skin- limited vs diffuse

A 
Limited :
Face, distal to knees and elbows
CREST – sclerodactyly
No internal organ fibrosis
Associated with pulmonary                                                                                    hypertension
Diffuse:
Proximal extremity
Trunk
Internal organ fibrosis
Interstitial lung Disease
Worse Prognosis

Early on skin can be edematous and inflamed. This early disease is often associated with pruritus. Dermal fibroblasts overproduce extracellular matrix that leads to increased tissue collagen deposition in the skin. Collagen cross-linking then causes progressive skin tightening

21
Q

Scleroderma Skin later stages

A

Later Stages are associated with atrophy, dry, and scaly skin. Pruritus continues with excoriations and thickening of the skin.
Skin ulcers over contractures
Digital ischemic ulcers in 30-50% of patients
Pitted areas of distal fingers with loss of digital pad
Telangiectasias of face and palmer surface of hands
Subcutaneous calcinosis in small percentage of patients

22
Q

Scleroderma and Raynaud phenomenon

A

First disease manifestation in most scleroderma patients
Develops concurrently with other symptoms in diffuse disease
Precedes other symptoms by years in limited skin disease
Stress and cold temperature lead to an exaggerated vasoconstriction of small arteries, arterioles, and arteriovenous shunts or thermoregulatory vessels of the skin of the digits.
Pallor, cyanosis, and rubor
Often painful in scleroderma and can lead to digital ulcerations, gangrene, or amputation
Patients over the age of 30 with Raynaud phenomenon should be screened with an ANA and nailfold capillary examination

23
Q

Scleroderma Lung Disease

A

Interstitial fibrosis secondary to an inflammatory alveolitis

  • Early diffuse scleroderma in the first 4 years
  • Predictors of progression
  • – Diffuse skin disease
  • – African American Race
  • – Early decline in FVC
  • – Fibrosis on CT
  • – Antitopoisomerase I antibodies (Anti-Scl-70)

Pulmonary Hypertension
- Occurs in longstanding disease

24
Q

testing for scleroderma lung disease

A

High Resolution CT Scanning
- Detects early pulmonary fibrosis

Pulmonary Function Testing

  • Detect changes in FVC over time
  • 80% of patients with scleroderma have restrictive disease
  • 10-20% suffer from progressive severe interstitial lung disease

Echocardiography

  • 25% of scleroderma patients have pulmonary hypertension
  • 10-15% have severe pulmonary hypertension
25
Q

Physical Findings and EKG findings of Pulmonary Hypertension

A 
Prominent P2
Right ventricular heaves
Hepatomegaly
Lower extremity edema
EKG
- Right axis deviation
P-pulmonale
- RBBB
- R/S > 1 in lead V1
26
Q

Scleroderma and GERD with dysphagia

A

Food getting stuck in esophagus, atypical chest pain, or cough
Have to drink liquids to swallow meat or bread
Secondary to smooth muscle atrophy
Untreated can lead to esophagitis, esophageal ulceration, esophageal stricture, or Barrett esophagus
Mucosal A-V malformations or telangiectasia in the wall of the stomach can lead to bleeding. GAVE (gastric antral vascular ectasia)

27
Q

Scleroderma GI

A 
Small and Large Intestines:
Smooth muscle atrophy
Constipation alternating with diarrhea
Pseudo-obstruction
Pneumatosis coli intestinalis
Bowel distention with leakage of air into the bowel wall
Bowel Rupture
Dysmotility
Bacterial overgrowth leading to diarrhea and malabsorption
Fecal incontinence
28
Q

Scleroderma Renal

A

Scleroderma Renal Crisis
5% of patients
Sudden onset of malignant hypertension
Untreated can lead to rapid renal failure and death

Risk factors:

  • Treatment with glucocorticoids
  • Anti RNA-polymerase III antibodies
  • Diffuse scleroderma

Symptoms
- Headache, visual changes, or seizure

Renal biopsy with intimal hyperplasia and vasospasm of the cortical arteries
- Leads to activation of renin-angiotensin system and accelerated hypertension, proteinuria, microscopic hematuria, and thrombotic microangiopathy (schistocytes on peripheral blood smear)

29
Q

Scleroderma Cardiac

A

Morbidity primarily in late stages of scleroderma
Microvascular disease and ischemia
Arrhythmias, cardiomyopathy, or overt CHF
Pericardial effusion frequently detected – usually clinically silent

30
Q

Scleroderma Musculoskeletal

A 
Arthralgias
Erosive arthritis
Joint contractures
Tendon friction rub
- Ankles, wrists, and knees
- Associated with increased disease activity and poor prognosis
Weakness
31
Q

Scleroderma Laboratory

A

ANA with 95% sensitivity, but not specific

Anticentromere antibodies

  • 20-40% of patients with scleroderma
  • Associated with limited skin involvement, more severe digital ischemia, and pulmonary hypertension (CREST)
  • Not specific to scleroderma
  • Also associated with primary biliary cirrhosis and Sjögren syndrome

Antitopoisomerase I (anti-Scl-70) antibodies

  • 20-40% of patients with scleroderma
  • Diffuse skin changes, interstitial lung disease, and worse prognosis

Anti-RNA Polymerase I/III antibodies
- Rapidly progressive diffuse skin changes, higher risk or renal involvement, lower risk of interstitial lung disease

32
Q

Scleroderma Differential Diagnosis

A

Scleredema

  • Poorly controlled diabetics, monoclonal gammopathies, or post-infectious (Streptococcal pharyngitis
  • Upper back, proximal extremities, and mid-portion of back (spared in scleroderma)
  • Autoantibodies absent

Scleromyxedema

  • Mucin deposition in the skin with cobblestone texture
  • Monoclonal gammopathy
  • Involves mid-back (spared in scleroderma), glabella, and ears
  • Neuro involvement in 10% of patients with seizures, global encephalopathy, and coma

Eosinophilic fasciitis

  • Involves deeper tissues and has a “woody texture”
  • Peau d’orange texture of skin
  • Eosinophilia
33
Q

Scleroderma Prognosis

A

Limited Scleroderma
- 90% 5 year survival

Diffuse Skin Disease
- 70-80% 5 year survival

Predictors of poor outcome

  • High skin scores
  • Progressive lung disease
  • Tendon friction rubs
  • Evidence of heart disease
  • Pulmonary artery hypertension
  • Anemia
  • Scleroderma Renal Crisis
34
Q

Dermatomyositis and Polymyositis

A

Peak ages 45-60 years

Symmetric proximal muscle weakness evolving over weeks to months

  • Difficulty rising from a low chair
  • Difficulty walking up stairs
  • Trouble washing or combing hair

Severe cases

  • Weakness of neck flexors
  • Pharyngeal weakness
  • Diaphragmatic weakness
35
Q

Dermatomyositis and Polymyositis Extramuscular Manifestations

A

Systemic symptoms: fever, malaise, weight loss, arthralgia, and Raynaud phenomenon
Joint Contractures
Dysphagia
Cardiac disturbance: AV defects, tachyarrhythmias, dilated cardiomyopathy, low ejection fraction, and CHF
Dyspnea secondary to diaphragm weakness or interstitial lung disease
Subcutaneous calcifications
Arthralgias, synovitis, or deforming arthropathy

36
Q

Dermatomyositis and Polymyositis Physical examination

A

Weakness of proximal arm muscles and Hip Flexors

  • Deltoids, but often biceps and triceps
  • Hip Flexors, but hamstrings and quadriceps can be weak
  • Distal muscle weakness only with very severe proximal weakness
Skin:
Gottron papules
Photosensitive rash – Shawl Sign or V-sign
Mechanic hands
Periungal telangiectasias
37
Q

Dermatomyositis and Polymyositis Lab findings

A

Creatinine kinase, aldolase, AST, and LDH elevations after release from damaged muscles
Anti-Jo-1 (aminoacyl tRNA synthetase) elevated in 30 % of patients
- 50-75% will have interstitial lung disease
- One of many synthetase autoantibodies

Antisynthetase antibody positive patients may have one or more of the following clinical features

  • Interstitial lung disease
  • Nonerosive arthritis
  • Fevers
  • Raynaud phenomenon
  • Mechanics hands

Antisynthetase syndrome – Antisynthetase antibody + 2 or more clinical features

38
Q

Dermatomyositis and Polymyositis MRI imaging

A

Most useful for evaluation of muscle edema as an indicator of active inflammation.
Can detect areas of patchy involvement to locate a good biopsy site
Useful to differentiate between active myositis and glucocorticoid-induced myopathy
- Edema is indicative of ongoing inflammation
Useful to monitor disease progression or response to treatment

39
Q

Dermatomyositis and Polymyositis Nerve Conduction Testing and EMG Testing

A

Detects whether weakness is due to a defect in the anterior horn cell, nerve, neuromuscular junction, or muscle

40
Q

Dermatomyositis and Polymyositis Muscle Biopsy

A

Should be performed in all patients except for those with the pathognomonic skin findings of dermatomyositis

41
Q

Dermatomyositis and Malignancy

A

Malignancy incidence increased in Dermatomyositis

  • ovarian cancer
  • breast cancer
  • melanoma
  • colon cancer
  • non-Hodgkin’s lymphoma

These patients should have complete annual physicals with pelvic, breast, and rectal examinations.

Mammograms and Colonoscopies as indicated

42
Q

Dermatomyositis and Polymyositis Treatment

A

Glucocorticoids
Glucocorticoid sparing agents
- Methotrexate
- Azathioprine

MSK II Exam 2 (16 decks)

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