Principales dominios

Departments of Cardiology and Medicine, GB Pant Hospital and University College of Medical Sciences, New Delhi

A

arteriopathy involving the aorta, its major branches and sometimes the pulmonary arteries. Involvement of the arch vessels is common. Stenosis and occlusion of the subclavian, carotid and vertebral arteries in various combinations are responsible for the multiplicity of cerebral and visual disturbances.1 _{However, its association with}

hypoparathyroidism has not been reported. We report the case of a young Indian woman who presented with hypoparathyroidism and aortoarteritis.

Case Report

A 25-year-old female presented with a history of generalized bone pain, episodes of muscle cramps and carpopedal spasm, along with decreased visual acuity for the last eighteen months. The patient had a history of one short episode of syncope. She also had constitutional symptoms with low-grade intermittent fever and loss of appetite and weight for the same duration of time. There was no history of pulmonary tuberculosis or neck surgery.

On physical examination, she had mild pallor and jugular venous pulsations were normal. Carotid and upper limb pulses were not palpable while lower limb pulsations could be well palpated. Blood pressure in the lower limbs was 150/ 80 mmHg and no bruit was audible. Examination of the cardiovascular system, chest, abdomen and central nervous system did not reveal any abnormality. Chvosteck’s sign and Trousseau’s sign were present. Both eyes had an immature cataract and the vision was 6/60. A clinical diagnosis of aortoarteritis with hypoparathyroidism was made.

Her hematological parameters revealed a raised ESR (130 mm fall in the first hour) while the total and differential leucocyte counts were normal. Biochemical parameters such as liver and kidney functions, and serum electrolytes were within normal limits. The level of serum phosphorus was high (6.6 mg/dl) while serum calcium (4.6 mg/dl) and serum parathormone (8.2 ng/dl) levels were low. The Mantoux test was positive (16×16 mm). The electrocardiograph (ECG), chest X-ray, skull X-ray, and X- ray of the hands, feet and pelvis were normal. Echocardiography was also normal, and a peripheral Doppler study revealed involvement of the carotid and subclavian arteries. The lower limb arteries were normal.

Diagnostic angiogram was done via the right femoral artery under local anesthesia. An arch aortogram revealed total occlusion of both the common carotid and both the subclavian arteries. Late frames showed collateral circulation to the vertebral arteries (Fig. 1). An abdominal aortogram revealed normal renal arteries and aorta. Pulmonary artery angiogram revealed normal pulmonary arteries. A diagnosis of Type 1 aortoarteritis with hypoparathyroidism was made and the patient was started on steroids (in view of activity), oral calcium gluconate and calciferol (Vitamin D₃) and put on regular follow-up. A follow-up angiographic re-study done after five years showed that all the arch arteries continued to be occluded while the abdominal aorta and its branches were spared. Serum parathormone (7.2 ng/dl) and serum calcium (6.1 mg/dl) levels continued to be low.

Discussion

Signs and symptoms in patients with Takayasu’s arteritis

Brief Report

Clinical manifestations of aortoarteritis (Takayasu’s arteritis) are varied, depending on the involved segment of the aorta and its branches. A case of a young Indian woman with aortoarteritis presenting primarily with hypoparathyroidism is reported. Aortogram showed total occlusion of the arch arteries. To the best of our knowledge, the occurrence of hypoparathyroidism in aortoarteritis has not been reported. Possible mechanisms of such an involvement are discussed. (Indian Heart J 2001; 53: 348–349)

Key Words: Aortoarteritis, Takayasu’s arteritis, Hypoparathyroidism

Correspondence: Dr Sanjay Tyagi, Professor of Cardiology, GB Pant

Hospital, New Delhi 110023

Indian Heart J 2001; 53: 348–349 Tyagi et al. Aortoarteritis Presenting with Hypoparathyroidism 349

(aortoarteritis), a disease of worldwide distribution and geographic variations, can show considerable clinical variation with regard to severity, duration and quality.2 _The

onset of the disease is often insidious and it progresses at a variable rate from the active inflammatory phase to the chronic, sclerotic phase with intimal hyperplasia, medial degeneration and adventitial fibrosis.3 _{Stenotic lesions are}

more common than aneurysms.4 _{The narrowing starts near}

the orifice of the artery and extends for a variable distance; progressive narrowing leads to occlusion with ischemic symptoms. In such an extreme form of brachiocephalic arteritis, all or most of the arch vessels are occluded and the entire circulation to the brain is provided through collateral vessels. Peculiarly, this case presented with symptoms of hypoparathyroidism.

Hypoparathyroidism is most commonly seen following surgery for thyroid disorders, hyperparathyroidism and radical dissection of the neck for cancer.5 _{Very rarely, it}

follows X-ray irradiation of the neck or massive radioactive iodine administration for cancer of the thyroid. To the best

of our knowledge, it has not been described in patients with aortoarteritis. Hypoparathyroidism in this case could be because of the compromised vascular supply to the parathyroids or a common autoimmune process involving them.6 _{A chance association of idiopathic}

hypoparathyroidism cannot be ruled out. Rare cases of ulcerative colitis with Takayasu’s aortitis have been reported and common immunological mechanisms have been postulated but not proved.7 _{An immunological study from}

India of 50 patients with aortoarteritis did not find any role of an autoimmune mechanism in aortoarteritis.8

Interestingly, the patient’s symptoms improved following medical treatment with steroids, calcium and vitamin D₃, but angiographic improvement on adequate, long-term steroids, as reported by some workers,9 _{was not seen in this}

case. No improvement of arterial obstruction has been reported in other studies.10 _{Kerr et al.}4 _{reported a relapse in}

half the patients who had initially achieved remission on steroids.

References

1. Lande A, Bard R, Bole P, Guranaccia M. Aortic arch syndrome (Takayasu’s arteritis)—arteriographic and surgical considerations. J Cardiovasc Surg 1978; 19: 507–613

2. Ishikawa K. Patterns of symptoms and prognosis in occlusive thromboaortopathy (Takayasu’s disease) J Am Coll Cardiol 1986; 8: 1041–1046

3. Sekiguchi M, Suzuki J. An overview on Takayasu’s arteritis. Heart Vessels 1992 (7 suppl); 6–10

4. Kerr GS, Hallanhan CW, Giordano J, Leawitt RV, Fauci AS, Rottem M, et al. Takayasu’s arteritis. Ann Intern Med 1994; 120: 919–929 5. Rude RK. Hypocalcemia and hypoparathyroidism. Curr Ther

Endocrinol Metab 1997; 6: 546–551

6. Hofbauer LC, Heufelder AE. Antibodies targeting the calcium sensing receptor: acquired hypoparathyroidism—an autoimmune disease at last? Eur J Endocrinol 1996; 135: 172–173

7. Ikenaga H, Ogihara T, Iyori S, Kou S, Yoshikawa H, Okura MM. Does a common pathophysiological basis exist in the association of ulcerative colitis and Takayasu’s aortitis?—report of a case. Postgrad Med J 1989; 65: 761–764

8. Chopra P, Datta RK, Dasgupta A, Bhargava S. Immunological studies in aortoarteritis. Indian J Med Res 1982; 76: 436–443

9. Ishikawa K. Effects of prednisolone therapy on arterial angiographic features in Takayasu’s disease. Am J Cardiol 1991; 68: 410–413 10. Morales E, Pineda C, Martinez-Lavin M. Takayasu’s arteritis in

children. J Rheumatol 1991; 18: 1081–1084

Fig. 1. Intra-arterial digital subtraction angiogram of the arch of aorta

showing total occlusion of the left subclavian, left common carotid, right common carotid and right subclavian arteries at their origin. Late frames showed collateral circulation to the vertebral arteries.

350 Ghadage et al. Infective Endocarditis due to Salmonella Indian Heart J 2001; 53: 350–351

Infective Endocarditis due to an Unusual