GRAND ROUNDS. Pulse For The Pulseless 40 CLINICAL PROCEEDINGS ANJANA GOPAL, SHIJI P V, CHANDNI R ABSTRACT HISTORY

Transcription

1 GRAND ROUNDS ANJANA GOPAL, SHIJI P V, CHANDNI R ABSTRACT Takayasu s arteritis is characterized by stenosis and inflammation of large and intermediate-sized arteries with frequent involvement of the aortic arch and its branches. Venous thrombosis in Takayasu s arteritis is rarely described. Inflammation-induced thrombosis is now considered a feature not only of autoimmune rheumatic diseases, but also of systemic vasculitides such as Behçet s syndrome, ANCA-associated vasculitis or giant cells arteritis, especially during active disease 1. Here is a 20 year old female, who presented with features of congestive cardiac failure, anemia and asymmetrical pulses which later turned out to be Takayasu s arteritis. Keywords: Takayasu s arteritis. HISTORY Our patient was a 20 year old female from Tribal community of Wayanad, presented with retrosternal heaviness and palpitation. She had dyspnea on exertion of 1 month duration. It was gradually progressive of NYHA (New York heart association) class II initially then progressed to class III. There is history of paroxysmal noctural dyspnea and orthopnea. She also gives history of pedal oedema of one month but no history of facial puffiness or abdominal distension. There was no history of loss of weight or syncope.there was no ascociated history of abdominal pain or altered bowel habits.no history of fever, joint pains or sore throat. No history of recurrent respiratory infections in childhood. No history suggestive of rheumatic fever in childhood. No history of diabetes, hypertension, tuberculosis, cardiac disease, Pulse For The Pulseless Anjana Gopal, Junior resident Shiji P V, Assistant professor Chandni R, Additional professor, Department of General Medicine, Govt Medical College, Kozhikode 40 CLINICAL PROCEEDINGS

2 bronchial asthma.no history of contact with tuberculosis. She gives history of amenorrhea for the past 3 month. There is also history of first trimester abortion 1 year back. Her diet was deficient in fruits and vegetable. On examination, she had pedal oedema and pallor.she was moderately built and poorly nourished.pulse rate of 120 beats per minute, regular, low volume with pulse asymmetry. Left radial, brachial, carotid was feeble compared to right.lower limb pulses were normal. No radio-femoral delay. No thickening of vessel wall. Bruit was heard over the left carotid and subclavian artery. There was asymmetry in blood pressure in the right and left upper limb. And there was difference in blood pressure between upper limb and lower limb. On examination of the cardiovascular system, there were features of pulmonary artery hypertension. Examination of respiratory system revealed bilateral basal crepitations suggestive of pulmonary oedema. After history and clinical examination, our differential diagnosis were 1.Takayasu s arteritis- in view of the asymmetric pulses and blood pressure. The presence of pulmonary artery hypertension could be due to pulmonary vasculitis or due to an ischemic cardiomyopathy due to coronary ostial involvement causing a congestive cardiac failure. 2. Anemia possibly sickle cell disease (being common in her geographic area), causing congestive cardiac failure and pulmonary artery hypertension. 3. Antiphospholipid antibody syndrome, because of the age, female sex, history of abortion and possibly chronic pulmonary thromboembolic disease causing pulmonary artery hypertension. With these differential diagnosis in mind, we proceeded with investigation. (table 1) Haemoglobin 8.7 WBC count Platelet count ESR 55 RBS 95 Blood urea 19 Serum creatinine 1 ALT 176 AST 102 ALP 123 Sodium/potassium 133/4 Table 1 Since the patient had low haemoglobin, we needed to workup for the cause of anaemia.peripheral smear was showing microcytic and hypochromic RBC s with anisopoikilocytosis. WBC Count was normal with predominant neutrophils.there was mild thrombocytosis,possibly reactive.since it was a microcytic and hypochromic anemia, iron studies were sent and there was evidence of iron deficiency. The workup of sickle cell disease was negative. And to rule out autoimmune haemolytic anemia, direct coomb s test(dct) was sent and it came as positive. There was elevated lactate dehydrogenase (LDH) and reticulocyte count. But ANA and ANA profile was negative. Hence, there was evidence of iron deficiency anemia and autoimmune haemolytic anemia. (table2) As part of the workup for the cardiac failure, Chest X Ray was taken which showed bilateral pleural effusion, cardiomegaly with right ventricular type of apex and features of pulmonary oedema. Pleural fluid analysis showed a transudative type of fluid.an Echocardiography was done, which showed global left ventricular hypokinesia, severe pulmonary artery hypertension(pah), severe tricuspid regurgitation(tr), mild to moderate mitral regurgitation(mr),moderate to severe left ventricular and right ventricular dysfunc- CLINICAL PROCEEDINGS 41

3 tion and mild pericardial effusion. Hence we considered a possibility of cardiomyopathy due to coronary ostial involvement and ischaemic cardiomyopathy. RDW MCV S FERRITIN IRON TIBC TSAT HPLC LDH DCT CORRECTED RETI COUT ANA ANA PROFILE NORMAL 787 POSITIVE 2.5% NEGATIVE NEGATIVE Table 2 We proceeded with the workup of Takayasu s arteritis. The erythrocyte sedimentation rate was 55 and CRP was 33 (normal <0.6). Doppler study of both upper limbs showed wall thickening involving both common carotid and internal carotid artery (left more than right ), left subclavian, axillary, radial and ulnar arteries,possibly large vessel vasculitis.mr aortogram showed diffuse wall thickening in the aorta measuring 3mm in ascending, 4mm at arch, 6.4mm in descending thoracic aorta, 5.2mm in abdominal aorta. Brachiocephalic trunk showing circumferential wall thickening at the origin. Complete thrombosis of left common carotid and its branches. Complete thrombosis of left internal jugular vein, brachiocephalic and subclavian veins.bilateral renal arteries showed beaded appearance with narrowing at its origin. Since the patient was having features of Takayasu s arteritis, workup for pulmonary tuberculosis, was also done. Sputum AFB, sputum gene Xpert and mantoux was negative. 42 Figure 1 and 2: showing the MR aortogram showing aortic wall thickening Figure 1 Figure 2 According to the ACR criteria for diagnosis of Takayasu s arteritis, we need to fulfil three out of the six criteria which includes: CLINICAL PROCEEDINGS

4 -Age at disease onset d 40 years, -Claudication of the extremities, -Decreased pulsation of one or both brachial arteries, -Difference of at least 10 mmhg in systolic blood pressure between the arms, -Bruit over one or both subclavian arteries or the abdominal aorta, and -Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia, or other causes. Our patient satisfied, five out of the six criteria, hence the diagnosis of Takayasu s arteritis was made. But some points still needed clarification. Why is there venous thrombosis in a patient with Takayasu s arteritis? Why does the patient have autoimmune haemolytic anemia? Since venous thrombosis was unusual in Takayasu s arteritis, a workup for thrombophilias was done. Since the patient also gave a history of abortion, a possibility of antiphospholipid antibody syndrome was considered. Antiphospholipid antibody, â 2 microglobulin and anticardiolipin antibody was done and it was negative. Antithrombin III, Factor V leiden, Protein C, Protein S was also done which were negative. In view of the arterial and venous involvement, workup of Behcet s was also done. It was also negative. Our next question was, why autoimmune haemolytic anemia? A workup for Systemic lupus erythematosis was done. ANA IF (immunofluorescence), and ANA Profile was negative. So we considered the existence of an underlying autoimmune disease in evolution. We followed up the patient and a repeat thrombophilic workup was done after 6 weeks. These were also negative. Hence we attributed the arterial and venous thrombosis to the underlying disease process of Takayasu s arteritis. We started the patient on steroids and anticoagulation with warfarin. The patient showed improvement. DISCUSSION Takayasu s arteritis is a chronic arteritis of unknown etiology. Women are affected in 80 to 90 percent of cases, with an age of onset that is usually between 10 and 40 years 2. Takayasu s arteritis primarily affects the aorta and its primary branches. The inflammation may be localized to a portion of the thoracic or abdominal aorta and branches, or may involve the entire vessel. The inflammatory processes cause thickening of the walls of the affected arteries. Narrowing, occlusion, or dilation of involved portions of the arteries in varying degrees results in a wide variety of symptoms 3. Venous thrombosis in Takayasu has been rarely described in literature. We found only 2 case reports of venous thrombosis. One case of superior vena cava thrombosis and other of cerebral venous sinus thrombosis. We presume that the thrombosis may be part of the vessel wall inflammation. Since the workup for thrombophilic states and behcets were negative, we assume that thrombosis was part of the underlying disease process of Takayasu. The existence of autoimmune haemolytic anemia also make this case unique. PEARLS 1. Give four causes of asymmetric upper limb pulse? Atherosclerosis, takayasu, coarctation of aorta, aortic dissection 2. What s takayasu s arteritis? It is a large vessel vasculitis characterised by inflammation of aorta and its branches 3. Name few conditions which can predispose to arterial as well as venous thrombosis? APLAS, Behcet s disease, hyperhomocystiemia, malignancies, paroxysmal nocturnal hemoglobinuria CLINICAL PROCEEDINGS 43

5 REFERENCES 1. Emmi G, Silvestri E, Squatrito D, Amedei A, Niccolai E, D Elios MM, DellaBella C, Grassi A, Becatti M, Fiorillo C, Emmi L, Vaglio A, Prisco D. Thrombosis in vasculitis: from pathogenesis to treatment. Thromb J Apr 16;13:15. doi: /s z. PubMed PMID: ; PubMed Central PMCID :PMC Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: new classification. Int J Cardiol 1996; 54 Suppl:S Sharma BK, Jain S, Sagar S. Systemic manifestations of Takayasu arteritis: the expanding spectrum. Int J Cardiol 1996; 54 Suppl:S CLINICAL PROCEEDINGS