• Takayasu arteritis

Takayasu arteritis is a chronic inflammatory vasculitis that occurs primarily in young women. It occurs world wide, with greatest prevalence in Asian people. It mainly affects the aorta and its major branches.¹ The Centres for Disease Control and Prevention have broadly defined idiopathic CD4+ T lymphocytopenia as a reproducible depletion of CD4 lymphocytes below 0.3×10⁹/l in the absence of HIV infection or other known causes of immunodeficiency.² We report a case of Takayasu arteritis with low CD4+ T lymphopenia without evidence of HIV infection in a boy from Turkey.

A 9 year old boy was admitted with a history of dyspnoea, malaise, and cough for four months. Before admission the patient had been prescribed treatment for pneumonia. He had no history of recurrent infection until four months before his admission. There was no parental consanguinity or any immunocompromised person in his family. Physical examination showed a temperature of 36°C, pulse rate of 140 beats/min, respiratory rate of 50/min, and a blood pressure of 110/70 mm Hg. His weight and height were below the fifth centile. He had a gallop rhythm, grade 3/6 pansystolic murmur at the 4th–5th left intercostal space and hepatomegaly. A chest x ray examination showed cardiomegaly and pulmonary oedema. The following laboratory values were obtained: haemoglobin 113 g/l, packed cell volume 0.35, leucocyte count 8.3×10⁹/l, platelet count 371×10⁹/l, erythrocyte sedimentation rate 71 mm/1st h. Other test findings, including serum electrolytes, blood urea nitrogen, and creatinine, were all normal. Echocardiography showed a dilated cardiomyopathy associated with severe mitral and aortic insufficiency. The patient was treated for heart failure with inotropic agents and furosemide (frusemide) and improved greatly.

At the fourth month of follow up a physical examination showed hypertension and decreased left radial and brachial pulses. A systolic blood pressure difference greater than 10 mm Hg between both arms appeared (right arm, 140/100 mm Hg; left arm, 110/70 mm Hg). Laboratory findings showed increased blood urea nitrogen and creatinine levels. Urine analyses disclosed microscopic haematuria and mild proteinuria. Antinuclear antibodies were positive (1/20). Protein electrophoresis showed a decreased serum albumin level, hypergammaglobulinaemia, and increased α₂ globulin. Serum C3 and C4 levels were normal. HLA-B52 was negative. Table 1 shows the immunological findings of this patient. A lymphocyte proliferation test was not carried out on the patient. Enzyme linked immunosorbent assay (ELISA) and polymerase chain reaction tests for HIV-1 and HIV-2 were repeatedly negative, but we did not look for HTLV-1 and HTLV-2. Echocardiography disclosed dilatation of the thoracic aorta and stenosis of the left subclavian artery. Magnetic resonance imaging showed dilatation and irregular contour of the ascending and descending aorta, and narrowing of the abdominal aorta (fig 1). The patient underwent cardiac catheterisation and aortography. Angiographic examination showed narrowing of the left subclavian artery, dilatation of the thoracic aorta, and occlusion of the superior mesenteric and renal arteries. Moreover, the patient's left kidney could not be visualised. Takayasu arteritis was diagnosed and the patient received prednisone treatment (2 mg/kg/day), but he died in the initial steroid treatment period owing to severe cardiac failure.

Takayasu arteritis may be the third most common form of childhood vasculitis after Henoch-Schönlein purpura and Kawasaki disease. Raised immunoglobulin levels and the finding of anti-aorta antibodies in the serum of some patients with this condition have suggested an immunological cause and, possibly, an autoimmune process.³

Low CD4+ T lymphocyte counts are rare in the absence of immunodeficiency, most commonly infection with HIV.² In our patient, major histocompatibility complex class II deficiency was excluded by the expression of HLA-DR on peripheral blood lymphocytes. Serological testing for HIV infection was negative and, additionally, the patient had no risk factor for transmission of HIV infection or recent immunosuppressive treatment.

All patients with idiopathic CD4+ T lymphocytopenia need to be observed prospectively and tested after their opportunistic infections, or after their first CD4+ cell count less than 0.4×10⁹/l ,to determine the natural history of their infections and lymphocytopenia. Two recent preliminary reports suggest the presence of a retrovirus in affected patients, but conclusive evidence is lacking.^4–7 The investigations of cases of idiopathic depletion of CD4 + T lymphocytes indicate that they probably represent various disorders, and that in some cases, low CD4+ T lymphocyte counts may reflect transient responses to infections or other conditions such as autoimmune disorders.⁴ In patients with aorta arteritis, immunological investigations have shown a decrease in the titre of complement and phagocytic activity of neutrophil granulocytes, deep depression of T cell immunity, and hypergammaglobulinaemia.⁸ Wiskott-Aldrich and Takayasu arteritis have been reported previously.⁹ It is rare for patients to have both disorders and with this case report, we draw attention to this association. This case report suggests that low CD4+ lymphopenia may cause dysgammaglobulinaemia and autoimmunity syndromes such as Takayasu arteritis.

• Download figure
• Open in new tab
• Download powerpoint

Figure 1

Magnetic resonance imaging shows dilatation and irregular contour of the descending aorta and narrowing of the abdominal aorta.