ATYPICAL CLAUDICATION IN A KARATE KID

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ATYPICAL CLAUDICATION IN A KARATE KID

• Pierre McCabe R5
• Internal Medicine
• CHUS, Sherbrooke University, Quebec
• Ted Giles Clinical Vignettes Session CSIM
• November 5, 2005

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Clinical case

• 42 year-old man
• Refered for an aortic occlusion discovered on a
  CT-Scan done for microscopic hematuria

• PMHx
• 1984 (at 22yo) History of left leg ulcer
• 1988 (at 28yo) Diagnosis of HTN
• 1990 normal renal arteriography

• Family history
• Mother and Sister HTN
• Medications
• Inderal and Zestril
• Smoking Stop since 10y

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Clinical case
Physical exam
Present illness

• No typical claudication
• Fatigue in buttocks and thighs during intense
  exertion
• Such as Karate
• Rapidly relieved by rest
• Stable in the last 4 years
• No symptoms at rest
• No impotence
• No constitutional complaint
• Systemic symptoms review negative

• Good general condition
• BP 140/100 in both arms
• Neck, heart, lungs, abdomen N
• Vascular exam
• Carotid, upper extremities abdomen no murmur
• Absent femoral pulses without murmur
• Weak distal pulses

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Clinical case Laboratory data

• CBC and renal fonction normal
• PTT 108.7sec PT normal
• Lupus anticoagulant positive
• Anticardiolipin Ab IgG 103 GPL (gt90 highly )
• Anticardiolipin Ab IgM 13 MPL (gt10 weakly )
• ESR and CRP normal
• RF and ANA negative

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Clinical case Imagery

• Duplex US of lower limb
• Monophasic waves starting at the iliac arteries
• Ankle-brachial index R 0,67 L 0,73
• Duplex US of upper extremities and carotid was
  normal

• CT-Angio
• Complete occlusion of the infrarenal aorta
• Other arteries N
• Subclavian arteries N
• Pulmonary arteries N
• 1990 Normal renal arteriography

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CT-Angio
Progressive narrowing until complete stenosis of
the infrarenal aorta
Collaterals
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CT-Angio
Progressive narrowing until complete stenosis of
the infrarenal aorta
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CT-Angio
Circonferential thickening of aortic wall
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CT-Angio
Circonferential thickening of aortic wall
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CT-Angio
Complete occlusion of the infrarenal aorta
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Clinical case

• Summary
• 42 year-old man with a proximal claudication
  caused by a chronic aortic occlusion
• Known for HTN, without other cardiovascular risk
  factor
• With antiphospholipid antibodies (LA aCL)
• Without any inflammatory markers

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Objectives

• To review the differential diagnosis of aortic
  occlusion
• To be aware of the relation between aortic
  disease and the antiphospholipid antibody
  syndrome
• To review the role of positron emission
  tomography (PET) in the assessment of
  large-vessel vasculitis

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Differential diagnosis Aortic occlusion

• Aortitis
• Idiopathic
• Takayasu arteritis
• Giant cell arteritis
• SLE
• Rheumatoid arthritis
• Spondyloarthopathy
• Relapsing polychondritis
• Behcets disease
• Sarcoidosis
• Infectious aortitis (syphilis, TB...)
• Antiphospholipid syndrome (APS)

• Atherosclerotic disease
• Thromboembolic disease
• Arterial thrombosis
• Antiphospholipid syndrome (APS)
• Other hypercoagulable states (myeloproliferative
  disorders, nephrotic syndrome, TTP/HUS)
• Fibromuscular dysplasia
• Retroperitoneal fibrosis
• Buergers disease
• Congenital hypoplasia

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Clinical case

• Differential diagnosis
• Active aortitis ?
• Large vessel vasculitis ? (Takayasu arteritis)
• PET scan No hypermetabolism in the
  large-vessel wall

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Clinical case

• Differential diagnosis
• Takayasu arteritis
• No other typical arteries affected
• Duplex US, CT-Angio and PET scan
• No history of inflammatory or systemic symptoms
• Antiphospholipid syndrome

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Antiphospholipid syndrome (APS)

• Definition
• Presence of antiphospholipid antibodies
• (Lupus anticoagulant, anticardiolipin Ab and
  anti-ß2 GP1 Ab)
• History of venous or arterial thrombosis, or an
  obstetrical complications (fetal loss,
  preeclampsia)
• Classification of APS
• Primary
• Secondary (associated with autoimmune disease)

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APS Associations

• APS and aortic occlusion
• APS and active aortitis

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APS and aortic occlusion

• Rare complication lt 1
• Kaushik S. Radiology 2001
• Physiopathology unknown
• Acute or subacute thrombosis
• Post-aortitis occlusion
• Premature atherosclerosis

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APS and aortic occlusion

• Synthesis of 8 cases
• 7 women et 1 man between 22 46 years
• Clinical presentation
• Infrarenal aortic occlusion 6/8 (75)
• Acute 1/7 vs Subacute Chronic symptoms 6/7
  (85)
• Other clinical manifestation compatible with APS
  100
• Pathology (2 cases)
• Intimal narrowing, no vasculitis and no
  atherosclerosis

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APS and active aortitis

• 2 cases reported Seror O. Radiol 1998
• Men of 45 and 69 years
• Abdominal and lower back pain, Fever, ESR
  elevated
• Antiphospholipid antibodies present
• Imagery
• Thickening of the aortic wall with infiltration
  of the periaortic fat
• Excellent response to steroid

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PET scan and vasculitis

• Aortitis and large-vessel vasculitis
• Angiography gold standard
• MRI-Angio
• Permits detection of earlier changes
• Poor correlation in follow-up with inflammatory
  markers
• PET scan (small studies)
• Diagnostic sensibility similar to MRI
• Seems more accurate for the follow-up
• Excellent correlation with inflammatory markers
  (ESR,CRP)
• False positive cause by moderate uptake with
  atherosclerosis
• Large clinical trials are necessary

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Back to the case

• Summary
• 42 year-old man with a distal aortic occlusion
• With antiphospholipid antibody (LA aCL)
• Without others manifestations of APS
• Without past or active vasculitis
• Diagnosis Infrarenal aortic occlusion possibly
  caused by a primary APS (1st manifestation)
• Treatment Warfarin (INR 2-3)

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Take home messages

• In case of arterial occlusion think of APS or
  large-vessel vasculitis (Takayasu or Giant cell
  arteritis)
• Aortic occlusion and aortitis are rare
  complications of APS
• The PET scan might have a new role in the
  diagnosis and follow-up of large-vessel vasculitis

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Références

• Alfayate JM. Aortoiliac thrombosis in
  antiphospholipid syndrome Case report and
  literature review. Vasc Endovasc Surg
  200235311-15.
• Soubrier M. Aortic occlusion in a patient with
  antiphospholipid antibody syndrome in systemic
  lupus erythematosus. Int Angiol 199514233-5.
• DiCenta I. Occlusion of the aorta and inferior
  vena cava in a patient with circulating
  anticoagulants. Ann Vasc Surg 200216380-3.
• Spronk PE. Severe atherosclerotic changes,
  including aortic occlusion, associated with
  hyperhomocysteinaemia and antiphospholipid
  antibodies. Ann Rheum Dis 2001 60699-701.

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Références

• Seror O. Aortitis with antiphospholipid
  antibodies CT and MR findings. Radiol
  199881373-5.
• Kaushik S. Abdominal thrombotic and ischemic
  manifestations of the antiphospholipid antibody
  syndrome CT findings in 42 Patients. Radiology
  2001218768-71.
• Scheel AK. Diagnosis and follow up of aortitis in
  the elderly. Ann Rheum Dis 2004 631507-1510.
• Schmidt WA. Use of ultrasonography and positron
  emission tomography in the diagnosis and
  assessment of large-vessel vasculitis. Curr Opin
  Rheumatol 2004179-15.

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Takayasu arteritis

• Definition
• Large-vessel vasculitis affecting the aorta and
  its primary branches
• Epidemiology
• Woman in 80-90 of cases
• Age of onset between 10 and 40 years
• Greatest prevalence in Asians
• Long term sequel
• Anevrismal dilatation, narrowing, occlusion

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Takayasu arteritis

• Diagnosis criteria (ACR) if 3
• Age at disease onset 40 years
• Claudication of the extremities
• Decreased pulsation of one or both brachial
  arteries
• Difference of 10mmHg in systolic BP between
  arms
• Bruit over subclavian arteries or the abdominal
  aorta
• Arteriography narrowing or occlusion of the
  entire aorta, its primary branches, or large
  arteries in the proximal UE or LE, not due to
  arteriosclerosis, fibromuscular dysplasia, or
  other causes

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Takayasu arteritis

• Clinical manifestations
• 1st stage
• Systemic gt Vascular symptoms
• 2nd stage
• Vascular symptoms
• Claudication UE LE, neurologic symptoms, angina
  MI, mesenteric ischemia, renovascular HTN
• 3rd stage
• Sequel resolution of the inflammation

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Takayasu arteritis

• Arteries affected
• Subclavian 93
• Thoracic and abdominal aorta 65
• Carotid 58
• Pulmonary 50
• Renal 38

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APS and Takayasu arteritis

• 4 cases of association described
• 2 cases Yokoik K. Angiology 1996
• Caso V. Cerebrovas Dis 2002
• Jean-Jaquet RS. Philipp J Obst Gynecol 1998
• No association found in 2 small studies
• Cohort of 28 patient (Nava a. Int J Cardiol 2000)
• Cohort of 21 patient (Girona E. Rev Invest Clin
  1993)

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Antiphospholipid syndrome (APS)

• Other clinical manifestations
• Thrombocytopenia 30
• Livedo reticularis 20
• Hemolytic anemia
• Cutaneous ulcer
• Livedoid vasculitis (atrophie blanche)
• Pyoderma-like gangrenosum
• Raynaud phenomenon
• Premature atherosclerosis

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TEP et Vasculite Suivi
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SAP Tx

• Traitement
• Coumadin
• Viser INR 2-3 gt 3-4
• Pas de différence de récidive (3.4 environ)
• Risque de saignement majeure semblable (2.2 vs
  3.6) (NEJM 20033491133)
• Durée ATC après un 1er épisode 12mois (grade 1C)
  ou indéfiniment (grade 2C)
• Durée ATC après une récidive à vie
• Ajout Aspirine pas de données claires
• Si thrombose artérielle, possible bénéfice
• Clopidogrel si all ASA
• Hydroxychloroquinine (Plaquenil)
• Possible bénéfice, surtout pour thrombose
  artérielle Très peu de données
• Prophylaxie (Prise en charge si pas ATCD MTE)
• ASA semble avoir un bénéfice
• Plaquenil possible bénéfice