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TAKAYASU’S ARTERITIS – a typical course of an untypical disease- a case report

TAKAYASU’S ARTERITIS – a typical course of an untypical disease- a case report. Anna Kapłańska Andrzej Łabyk Sławomir Tymiński Students’ Research Group, Department of Internal Medicine, Hypertension and Vascular Diseases, Warsaw Medical University. BACKGROUND. Takayasu arteritis (TA)

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TAKAYASU’S ARTERITIS – a typical course of an untypical disease- a case report

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  1. TAKAYASU’S ARTERITIS – a typical course of an untypical disease- a case report Anna Kapłańska Andrzej Łabyk Sławomir Tymiński Students’ Research Group, Department of Internal Medicine, Hypertension and Vascular Diseases, Warsaw Medical University

  2. BACKGROUND Takayasu arteritis (TA) • systemic inflammatory disease of unknown origin • affects primarily large vessels including aorta and its branches • due to variable clinical manifestations it is a diagnostic challenge

  3. AIM To present the variety of symptoms, typical course, complications( in connection with arterial involvement localization) and diagnostic difficulties in patient with TA

  4. METHOD • a case report • 30-year-old woman • follow-up – 1.5 year • type V P(+) of TA

  5. TYPES OF TA C(+) coronary arteries involvement P(+) pulmonaty arteries involvement „New angiografic classification of TA” TA conference, Angiology 1997; 48:369-79

  6. ACR CRITERIA FOR TA CLASSIFICATION • Age at disease onset <40 • Decreased brachial artery pulse (one or both) • SBP difference >10 mmHg (between arms) • Bruits over SAs / abdominal aorta • Arteriogram abnormalities • Claudication of extremities TA if present >= 3/6 Sensitivity 90%, specificity 98%

  7. RESULTSCLINICAL MANIFESTATIONS (1) On admission/ present complains • Haemoptysis • Pleuritic chest pain main reasons for admission • Chronic, non-productive cough • Large joints artralgia (ankle & knee) • Thoracic spinalgia • Progressive weakness

  8. CLINICAL MANIFESTATIONS (2) In anamnesis • Syncope ( twice, 2 years ago) • Claudication of R upper extremity • Heart palpitations (for many yrs) • Tachycardia • Pregnancy induced HA • HA after pragnancy ( 240/100 mmHg despite treatment) • Raised temperature ( for 3 months) • No PMH of altered visual acuity

  9. CLINICAL MANIFESTATIONS (3) On examination • Malnutrition • Pallor • Greyish nodules on forearms (similar to erythema nodosum) • Absent brachial & radial pulses • Murmurs over aorta, renal arteries, carotids, SAs • No retinal changes on fundoscopy

  10. CLINICAL MANIFESTATIONS (4) R BP L -/- mmHg ? 70/- mmHg 200/70 mmHg 195/80 mmHg

  11. LABORATORY TESTS ABNORMALITIES (1) ESR ↑ 195 mm/h (N: 3-15 mm/h) CRP 7.3 g/dl (N< 10 g/dl) WBC ↑ 15.0 G/l (N: 4-10.0 G/l) RBC ↓ 3.93 T/l (N: 4.2-5.5 T/l) Hbg ↓ 8.6 g/dl (N: 12- 14.0 g/dl) Hct ↓ 26.2 % (N: 37-47 %) MCV ↓ 66.5 fl (N: 81-99 fl) PLT ↑ 711 G/l (N: 150-400 G/l)

  12. LABORATORY TESTS ABNORMALITIES (2) Fibrinogen ↑ 736 mg/dl (N: 200-500mg/dl) IgG IgA in a normal range IgM IgE USR (-) HIV (-) HbS (-) HCV (-) cANCA(-)-ive → (+)-ive ( 35.82 U/l) pANCA(-)-ive → (+)-ive ( 36.38 U/l)

  13. LABORATORY TESTS ABNORMALITIES (3) Total protein 6.6 g/dl (N: 6-8.0 g/dl) Albumin ↓ 52.4 % (N: 60.3 - 71.4%) α-1 globulin ↑ 4.5 % (N: 1.4 -2.9 %) α-2 globulin ↑ 17.7 % (N: 7.2 – 11.3 % ) β-globulin ↑ 14.3 % (N: 8.1 – 12.7 % ) γ-globulin 11.1 % (N: 8.7 – 16 % )

  14. CHEST CT SCAN Consolidations in the upper & middle lobe of R lung ↓ Lung infarct

  15. Angio CT scan (1) moderate stenosis of L CCA Complete oclussion of R CCA Sign. stenosis of brachiocephalic trunk Sign. stenosis of L SA Dilatation of ascending aorta → strongly suggestive of TA

  16. Angio CT scan (2) Aortic aneurysm

  17. Angio CT scan (3) Dissecting aneurysm (?)

  18. Abdominal CT scan narrowing of truncus celiacus

  19. DOPPLER ULTRASOUND • Did not confirm dissecting aneurysm • Confirmed multiple arterial narrowings • Revealed bilateral steal syndrome R >> L • Renal arteries - bilaterally double but with normal blood flow

  20. ECHOCARDIOGRAPHY (transthoracic) • Global LV function- normal • No global/focal hipokinesia • Valves’ orifices and gradients- normal • Dilatatiom of ascending aorta • IAS aneurysm

  21. MAIN DIAGNOSTIC & THERAPEUTIC DIFFICULTIES • General symptoms & signs → the most common & the least specific → further carreful investigations • How and where to detect BP in TA pt? • How much can we reduce BP in TA pt? • Confusing imaging studies • PAs involvment → lung infarct → haemoptysis & chest pain • steal syndrome → impaired cerebral blood circulation → syncope

  22. CONCLUSIONS (1) • General symptoms of TA may be similar to other inflammatory diseases • Since there are no specific laboratory tests for TA, numerous imaging studies should be performed to confirm it

  23. CONCLUSIONS (2) • TA should be considered in pts with multiple arterial lesions despite its low prevalence • Symptoms and complications depend on the involvement of particular arteries and may consist of impaired cerebral blood flow, visual problems, HA, HP, pulmonary infart

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