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Finding a Hidden Giant

Finding a Hidden Giant. Rheumatology Case Presentation Registrar: JLeR Malherbe Consultant: C Nel. Presenting History. Mrs HL is a 68 year old lady from Odendaalsrus Reports that left radial pulse disappeared 4 months ago More recently her right radial pulse also disappeared

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Finding a Hidden Giant

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  1. Finding a Hidden Giant Rheumatology Case Presentation Registrar: JLeR Malherbe Consultant: C Nel

  2. Presenting History • Mrs HL is a 68 year old lady from Odendaalsrus • Reports that left radial pulse disappeared 4 months ago • More recently her right radial pulse also disappeared • She has bilateral arm claudication • No calf claudication

  3. Presenting History • No Headaches • No Jaw claudication • No Visual disturbances or loss of vision • She has lost weight and feels chronically tired • Reports no fever or nightsweats • She has palpitations • Pain of shoulder and neck muscles. Worse in morning. Can’t state a specific date of onset. Also reports pain around hips

  4. Previous History • No Hypertension or Diabetes • Only smoked for a few months when younger • No alcohol history • Multiple DVT’s. After surgery. Using warfarin for 12 years (episode early January INR suptherapeutic) • No History of miscarriages • Family history of genetic hypercoagulable state

  5. Clinical Examination • Did not look acutely or chronically ill • J- A- C- C- O- L- • Respiratory exam normal • Cardiac: Bigeminal rhythm, no murmers, no CCF • Abdominal: No HSM, no bruits • Very weak radial pulse in right arm • Absent radial pulse on left • No skin changes or ulceration but both hands cold

  6. Clinical Examination • Femoral and leg pulses normal • No pain over temporal arteries • No temporal artery thickening • Fundoscopy normal • Neurological examination normal

  7. Special Investigations • Duplex Doppler of arms  Occlusion with ?thrombus of left axillary artery and severe stenosis of distal right axillary artery • Arteriogram rigth arm  Complete right axillary artery stenosis at transition with brachial artery. Extensive collaterals. Complete stenosis of the radial artery at it’s origin. Interosseous and ulnar artery normal. Subclavian artery and aorta and other branches normal • Lupus anticoagulant positive

  8. Picture so far • Considering • Strong family history of hypercoagulability • Patient history of DVT’s • Episode of subtherapeutic INR (Although her symptoms started before this) • Positive Lupus Anticoagulant (Will need repeat in 12 weeks) Would be easy to attribute her presentation purely to the hypercoagulable state with arterial thrombosis/emboli BUT.........

  9. Rest of her Investigations • ESR > 90 (On multiple occasions) • CXR, CT abdomen, PEF excluded TB and occult malignancy • CRP 66  No evidence of infection • Hb 10.4, MCV 78.7 Plt 426, WCC 7.46, Eos 0.12 • Fe 3.4, TF 1.9, TF sats 7%, Ferritin 231 • RC Folate 529, VitB12 444 • Albumin 28, Rest of Biochem (U/E, LFT, CMP normal)

  10. Rest of her Investigations • ANCA  Negative • ANA  Negative • RF  Negative • ECHO/TEE  Normal heart with no source of emboli • ECG  Sinus rhythm with periods of bigeminy

  11. Thus • There’s clear evidence of systemic inflammation with anaemia of chronic inflammation and slightly low albumin • ? Vasculitis • Large vessel • Old patient ?GIANT CELL ARTERITIS?

  12. GCA  Pathogenesis • Chronic vasculitis of large and medium size vessels • Cranial branches of the arteries originating from the aortic arch. Can be generalized. Almost never intracranial arteries • Etiology and pathogenesis unknown • Increasing age, Genetic/Ethnic susceptibility (HLA-DR4), Infectious agents (Parvo B19) • Antigenic trigger  Activation monocytes  Inflammatory cytokines (systemic symptoms)  Infiltration of adventitia  More cytokines, lymphocytes and macrophages  chronic inflammation  destruction, fibrosis, thrombosis

  13. GCA  History • Age > 50 • Usually gradual onset. Can be abrupt • Systemic complaints  Fever, weigtloss, fatigue • New headache in 2/3  Often temporal • Jaw Claudication ½ (Specific) • Visual symptoms  Amaurosisfugax, AION, Diplopia, Bitemporalhemianopia, Homonymous hemianopia, Visual hallucinations • Polymyalgiarheumatica  40 – 50% of GCA has PMR, 5% of PMR has GCA • CNS  TIA, vertigo, hearing loss, and stroke

  14. GCA  History • 3 – 15% subclavian/axillary arteries sufficiently narrowed to cause Arm Claudication • Younger age of onset (66 vs 72) • Less likely to have headache (14% vs 57%) • Less likely to have temporal artery involvement Brack A; Martinez-Taboada V; Stanson A; Goronzy JJ; Weyand CM; Disease pattern in cranial and large-vessel giant cell arteritis: Arthritis Rheum 1999 Feb;42(2):311-7

  15. GCA  Examination • Thickened and tender temporal artery • Absent temporal pulse • Absent or diminished carotid, radial, femoral or pedal pulses • Bruits over major arteries • Aortic regurgitation murmer heralds development of ascending aorta aneurysm

  16. GCA  Diagnosis • Compatible clinical picture • Laboratory • ESR  Often > 100. Out of 941 biopsy proven GCA cases only 4 had normal ESR’s • CRP levels tend to parallel ESR. • Anaemia of chronic inflammation, Reactive thrombocytosis, Hypoalbuminaemia, Mildly elevated transaminases Smetana GW; Shmerling RH; Does this patient have temporal arteritis? JAMA 2002 Jan 2;287(1):92-101.

  17. GCA  Diagnosis • Biopsy of temporal artery (or other accessible clinically involved artery) should be performed on all suspected cases of GCA • If artery not clinically abnormal  at least 2cm. Shorter if visibly abnormal • Biopsy should be examined in multiple cuts • Consider bilateral biopsy (unilateral misses 7.4 to 13%) • Sensitivity of 85% in appropriate clinical setting • In phenotype clinically involving only subclavian or axillary artery sensitivity is 58% Brack A; Martinez-Taboada V; Stanson A; Goronzy JJ; Weyand CM; Disease pattern in cranial and large-vessel giant cell arteritis: Arthritis Rheum 1999 Feb;42(2):311-7

  18. Mrs HL’s Biopsy

  19. Mrs HL’s Biopsy

  20. GCA  Pathology • Vasculitic lesions involve arteries in patchy fashion • T lymphocytes and macrophages predominate • Variable numbers of multinucleated giant cells (50% of cases). Occasional plasma cells, neutrophils, and eosinophils. • Inflammation tends to occur mainly in media and internal elastic lamina (becomes fragmented) • Intimal thickening and thrombosis • Based on above Mrs HL’s biopsy was reported as being non-diagnostic for GCA BUT.... • In minimally involved arteries inflammation can be localized to adventitia/external internal elastic lamina

  21. GCA  Diagnosis • MRI/MRA • Especially suited for large vessels GCA • Can demonstrate early arterial wall oedema/thickening and inflammation • Localize temporal artery disease  Guiding biopsy • MR at Universitas does not have sufficient resolution • Conventional angiography • Only demonstrate lumen and not wall • Tapering of subclavian/axillary artery is typical

  22. GCA  Diagnosis • Ultrasound • Demostratestenosis, occlusions • Halo sign • Highly dependant on operator  sensitivity in predicting positive biopsy only 40%

  23. Considering the constellation of Mrs HL’s: • clinical signs and special investigations • Potential serious complications of untreated GCA (Visual loss, limb ischaemia) we decided to treat her as GIANT CELL ARTERITIS • Main differential diagnosis is Takayasuarteritis • Age • Arteries involved • Renovascular hypertension is common • Visual loss rare • Other vasculitides usually excluded on histology and vascular bed involvement

  24. GCA  Treatment • GCS is mainstay • Prednisone 40 to 60mg dly Taper slowly over a period of 9 to 12 months • IV methylprednisone pulse if visual loss • Low dose aspirin (plus PPI) • Prophylaxis against osteoporosis • ESR and CRP to monitor disease activity • Remember complication of aortic aneurysm

  25. Conclusion • This is an unusual presentation of GCA • Diagnosis confused by presence of a history of thrombophilia • Up to 18% of GCA may be positive for Lupus Anticoagulant/ACLA/B2GP but there’s no statistical correlation with thrombotic ischaemic events. Congenital thrombophilia does not have a higher incidence in GCA Espinosa G, Tàssies D, Font J, Muñoz-Rodríguez FJ, Cervera R, Ordinas A, Reverter JC, Ingelmo M; Antiphospholipid antibodies and thrombophilic factors in giant cell arteritis: Semin Arthritis Rheum. 2001 Aug;31(1):12-20

  26. References • Hunder GG; Pathogenesis of giant cell (temporal) arteritis; Clinical manifestations of giant cell (temporal) arteritis; Diagnosis of giant cell (temporal) arteritis; Treatment of giant cell (temporal) arteritis: UpToDate 17.3 • Brack A; Martinez-Taboada V; Stanson A; Goronzy JJ; Weyand CM; Disease pattern in cranial and large-vessel giant cell arteritis: Arthritis Rheum 1999 Feb;42(2):311-7 • Smetana GW; Shmerling RH; Does this patient have temporal arteritis? JAMA 2002 Jan 2;287(1):92-101 • Espinosa G, Tàssies D, Font J, Muñoz-Rodríguez FJ, Cervera R, Ordinas A, Reverter JC, Ingelmo M; Antiphospholipid antibodies and thrombophilic factors in giant cell arteritis: Semin Arthritis Rheum. 2001 Aug;31(1):12-20

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