No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
Celiac disease in 3 patients with Takayasu's arteritis
Abstract
Takayasu's arteritis (TA) is a chronic vasculitis of unknown etiology. Celiac disease (CD) is an autoimmune disease caused by the ingestion of gluten. TA and CD have been associated with many other autoimmune conditions. However, only five cases with this association have been reported. In this series, three patients with TA and CD were included; all were female, 21, 30 and 54 years old. TA clinical manifestations preceded CD diagnosis in all patients. Aortic arch branches were affected in all of them. Serologic markers were positive and a small intestine biopsy showed typical findings of CD in the three patients. Special attention should be given to this possible association because these entities may be asymptomatic; the recognition of new disease variants modifies treatment, and sometimes CD constitutes a differential diagnosis of mesenteric ischemia.
The clinical spectrum of celiac disease continues to evolve. What was once thought to be a rare disorder affecting young children is now recognized to be very common with a range of symptoms from asymptomatic disease to severely affected persons. Screening for celiac disease has become relatively easily with reliable antibodies against self-antigens (TG) and modified environmental antigens (DGP). Diagnosis is confirmed by small intestinal biopsy with characteristic changes graded by the Marsh score. Elimination of gluten from the diet has been the standard of care for the last half century. Patients often have difficulty adhering to a gluten-free diet, and the failure of symptoms, antibody levels, or pathologic changes to improve after initiating the diet may be largely due to this difficulty. The genetic risk for celiac disease is largely related to HLA genotypes, with over 90% of subjects with celiac disease positive for DQ2 and the remainder positive for DQ8. The HLA association with celiac disease is largely accountable for its link to other autoimmune diseases, including type 1 diabetes and autoimmune thyroid disease, and the majority of risk for celiac disease in these populations is related to HLA genotype. Celiac disease also carries an increased risk for type 1 diabetes and autoimmune thyroid disease. Genetic syndromes such as Turner and Down syndromes are associated with an increased risk for celiac disease. Practitioners can identify groups of subjects at high risk for celiac disease and perform screening with celiac disease-related antibodies.
Takayasu's arteritis is a non-specific form of vasculitis involving the aorta, its main branches and pulmonary arteries. It is a rare disease in our country, contrasting with the high prevalence in Southeast Asia, Africa and South America. We discuss the course of the disease in our patient who was a young women who developed Takayasu's arteritis associated with autoimmune thyroiditis and malabsorption syndrome due to celiac sprue. Long-term immunosuppressive therapy contributed to stabilizing the associated diseases, but did not stop the progression of the vascular lesions. The main adverse outcome in our patient was the development of severe renovascular hypertension. Bilateral renal artery stenosis was treated by angioplasty with stent implantation. Based on literature reports, the association of Takayasu's disease with multiple autoimmune disorders is a rare event. However, it would appear that the arteritis was the limiting disease for prognosis in our patient.
Giant-cell arteritis typically involves extracranial branches of the aorta, such as the temporal and vertebral arteries. Temporal arteritis and polymyalgia rheumatica often coexist, and systemic manifestations of inflammation occur in virtually all forms of giant-cell arteritis. This article reviews the mechanisms that contribute to the causes of giant-cell arteritis, with an emphasis on immune-mediated injury to arteries.
Midaortic syndrome is a variety of aortic coarctation, located in the distal thoracic aorta, the abdominal aorta or both, involving the intestinal and renal vessels, usually presenting with renovascular arterial hypertension. Underlying conditions are thought to be Takayasu's arteritis, von Recklinghausen's disease, and connate hypoplasia. Celiac disease is an inflammation in the small intestine, triggered by an allergic reaction to gluten. It is known to be associated with a variety of other autoimmune disorders, e.g., dermatitis herpetiformis (Duhring's disease), insulin-dependent diabetes mellitus, and IgA nephropathy. We describe the case of a young woman who presented with claudication of the lower limbs, therapy-refractory arterial hypertension, and untreated celiac disease. We found a midaortic syndrome, characterized by severe stenosis of the infrarenal aorta, of both renal arteries (more pronounced on the right side) and of the inferior mesenteric artery. We assume that-after having excluded other possible pathogeneses-the underlying condition is a local vasculitis in the abdominal aorta and the renal and mesenteric arteries due to the chronic inflammation of untreated celiac disease. We performed a percutaneous transluminal angioplasty together with implantation of two stents into the infrarenal aorta and the right renal artery and started treating the celiac disease by dietary intervention. The patient is now under regular medical control and observation.
Takayasu's arteritis (TA) is a rare vasculitis. The Italian Takayasu's Arteritis study group was established with the aim to describe a large cohort of patients.
Data were collected by means of an ad hoc form. Demographic information, clinical history, vascular findings, treatment, risk factors, and comorbidities were analyzed.
Data of 104 patients were collected. The median delay in diagnosis was 15.5 months (range 0-325 months). Age at onset <15 years was associated with a higher probability, whereas elevated erythrocyte sedimentation rate with a lower probability, of a delay in diagnosis. The majority of patients experienced nonspecific signs and symptoms indicative of an inflammatory disease in the early phase. Among vascular involvement, stenosis was the most frequent lesion, being present in 93% of patients, followed by occlusion (57%), dilatation (16%), and aneurysm (7%). Glucocorticoids were the mainstay of treatment in our series; however, treatment with cytotoxic agents was required in about half of the patients. Fifty-two patients underwent at least 1 surgical procedure. The main indications for intervention were renal vascular hypertension, cerebral hypoperfusion, and limb claudication.
As with many rare diseases, delay in diagnosis is an important issue for patients with TA. The increasing occurrence of vascular lesions along with the disease progression put to question the long-term effectiveness of contemporary treatment. These data may be helpful in increasing physicians' awareness to prevent diagnosis delay, update guidelines, and plan future research projects.
- Gs Kerr
- Cw Hallahan
- J Giordano
- Ry Leavitt
- As Fauci
- M Rottem
Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, et al. Takayasu
arteritis. Ann Intern Med. 1994;120:919–29.