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) systemic inflammatory disease of unknown origin systemic inflammatory disease of unknown origin affects primarily large vessels including aorta and its branches affects primarily large vessels including aorta and its branches due to variable clinical manifestations it is a diagnostic challenge due to variable clinical manifestations it is a diagnostic challenge

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

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

TYPES OF TA type involvement of the arteries I Branches from the aortic arch IIa Ascending aorta, aortic arch & its branches IIb Ascending aorta, aortic arch, its branches & thoracic descending aorta III Thoracic descending aorta, abdominal aorta &/or renal arteries IV Abdominal aorta &/or renal arteries V Combine features of types IIb and IV C(+) coronary arteries involvement P(+) pulmonaty arteries involvement „New angiografic classification of TA” TA conference, Angiology 1997; 48:369-79

ACR CRITERIA FOR TA CLASSIFICATION 1. Age at disease onset <40 2. Decreased brachial artery pulse (one or both) 3. SBP difference >10 mmHg (between arms) 4. Bruits over SAs / abdominal aorta 5. Arteriogram abnormalities 6. Claudication of extremities TA if present >= 3/6 Sensitivity 90%, specificity 98%

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

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

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

CLINICAL MANIFESTATIONS (4) R BP L -/- mmHg? 70/- mmHg 200/70 mmHg195/80 mmHg

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: G/l) RBC↓ 3.93 T/l (N: T/l) Hbg ↓ 8.6 g/dl (N: g/dl) Hct↓ 26.2 % (N: %) MCV↓ 66.5 fl (N: fl) PLT↑ 711 G/l (N: G/l)

LABORATORY TESTS ABNORMALITIES (2) Fibrinogen ↑ 736 mg/dl (N: mg/dl) IgG IgA in a normal range IgMIgE USR (-) HIV (-) HbS (-) HCV (-) cANCA (-)-ive → (+)-ive ( U/l) pANCA (-)-ive → (+)-ive ( U/l)

LABORATORY TESTS ABNORMALITIES (3) Total protein 6.6 g/dl(N: g/dl) Albumin ↓ 52.4 % (N: %) α-1 globulin ↑ 4.5 % (N: %) 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 % )

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

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

Angio CT scan (2) Angio CT scan (2) Aortic aneurysm

Angio CT scan (3) Dissecting aneurysm (?)

Abdominal CT scan narrowing of truncus celiacus

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

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

MAIN DIAGNOSTIC & THERAPEUTIC DIFFICULTIES 1. General symptoms & signs → the most common & the least specific → further carreful investigations 2. How and where to detect BP in TA pt? 3. How much can we reduce BP in TA pt? 4. Confusing imaging studies 5. PAs involvment → lung infarct → haemoptysis & chest pain 6. steal syndrome → impaired cerebral blood circulation → syncope

CONCLUSIONS (1) 1. General symptoms of TA may be similar to other inflammatory diseases 2. Since there are no specific laboratory tests for TA, numerous imaging studies should be performed to confirm it

CONCLUSIONS (2) 3 TA should be considered in pts with multiple arterial lesions despite its low prevalence 4 Symptoms and complications depend on the involvement of particular arteries and may consist of impaired cerebral blood flow, visual problems, HA, HP, pulmonary infart