Systemic Lupus Erythematosus with Takayasu’s Arteritis - A Rare Co-existance

We report the case of a 30-year-old normotensive, nondiabetic lady diagnosed case of Systemic Lupus Erythematosus(SLE); who came with the complaints of limb claudication. After clinical and lab evaluation she was diagnosed as a case of Takayasus Arteritis along with SLE. Though the co- existence of SLE and Takayasu’s Disease is very rare, we should search for the development of arterial occlusive diseases in SLE cases if the patient has got suspicious symptoms. J MEDICINE 2021; 22: 146-149

Revisiting the history of Takayasu's disease studies and surgical techniques used in its treatment

Nonspecific aortoarteritis is a systemic disease, which has been referred to the group of vasculitis affecting elastic and muscular arteries oflarge and medium calibre with the inflammatory processlocalized in the media and adventitia. The article presents the history of development of ideas about clinical manifestations, morphological changes and the course of the disease from 1761 to the present day, the timeline of medical advances in this disease studies. The genuine interest in nonspecific aortoarteritis arose at the beginning of thelast century, when the Japanese ophthalmologist Mikito Takayasu reported unusual changes in the retinal vessels of a 21-year-old Japanese girl suffering from recurrent bouts of syncope. The first publications dealt with clinical manifestations in the patients, who had only brachiocephalic arterial involvement. In the early 60s, it was found that nonspecific aortoarteritis (Takayasu's disease) can affect not only the branches of the aortic arch, but also the thoracic aorta, renal and visceral arteries. It was the mosaic clinical manifestations in patients with various forms of Takayasu's disease that caused the presentation of the disease in theliterature until the mid-1970s under various terms such as “pulseless disease”, “arteritis of young women”, “brachiocephalic arteritis”, “atypical coarctation of aorta”, “Martorell's syndrome”, “syndrome of obliteration of the supra-aortic trunks”, “panaortitis” or “panarteritis”, “aortitis syndrome”, “mid-aortic syndrome”, “occlusive thromboarteriopathy”. The review details the epidemiology and prevalence of this disease. Views not only on the etiology and pathogenesis, but also on the methods of treating this disease have changed since M. Takayasu's publication in 1908. Much attention is paid to the historical aspect of the first surgical procedures. Starting in 1951, the surgical method has firmly taken the lead in the treatment of stenosis of the carotid arteries, thoracic aorta, renal and visceral arteries. Surgical concepts changed, but the literature data indicate the sustainability of the basic principle of treatment: combination of surgical interventions and various antiinflammatory therapy regimens.

Nonspecific aortoarteritis (Takayasu’s disease)

The article presents a clinical case of Takayasu’s disease (nonspecific aortoarteritis). On the example of the course of the disease in a young woman, the features of clinical symptoms and diagnostic difficulties are analyzed. Particular attention is paid to the visualization of vascular lesions and the assessment of the dynamics of changes in the vessels as the disease progresses. Changes in laboratory parameters are presented in details according with ongoing therapy. The analysis of literature data of the effectiveness of drug therapy for the disease was carried out. It is emphasized that there is a very limited number of quality placebo-controlled studies in the literature. The practicing physician can only rely on the results of observational studies in his decision’s making process. Also, all studies presented in the literature included a very small number of patients. The algorithm of therapy prescription is given, the first line of which is glucocorticoids. Although there are very few literature data, it is noted that the achievement of remission with isolated glucocorticoid therapy is observed in 50 % of patients. According to the updated EULAR recommendations, after disease verification, therapy should be initiated immediately, the recommended starting dose is 40–60 mg of prednisolone (or equivalent). After achieving control of the disease, it is recommended to gradually reduce the dose to the target 15–20 mg/day for 2 to 3 months after 1 year to 10 mg/day. The next step is to prescribe a non-glucocorticoid immunosuppressant in order to provide both a «steroid sparing» effect and long-term control under the disease. All available information on the efficacy of methotrexate, mycophenolate mofetil, cyclophosphophomide, leflunamide, and azathioprine comes from open-label prospective or retrospective studies with a limited number of participants. Tumor necrosis factor inhibitors and tocilizumab are recommended as second-line drugs. A satisfactory clinical and laboratory response was observed in more than 80 % of patients receiving tocilizumab therapy. In this clinical case, tocilizumab was used and the effects of the drug during treatment for 8 months are described in detail. Immunobiological therapy with tocilizumab was associated with a good clinical and laboratory response, a decrease in vascular stenosis, and an improvement in the patient’s well-being.

Hypertension and Takayasu Disease

Introduction: Takayasu’s disease (TD) is a primary, inflammatory, rare, segmental and multifocal arteritis of the aorta and its branches, of unknown etiopathogenesis. Hypertension is a major complication, sometimes revealing the disease, whose mechanisms are multiple, dominated by the lesion of the aorta and renal arteries. Patients and methods: We report a series of 63 cases treated for Takayasu’s disease resulting from a monocentric, retrospective study from April 2oo6- July 2014, with an average age of 30 years with a female predominance of 83%. Result: In our series: 51 patients with hypertension, including 29 (57%) resistant hypertension (under tri see quadritherapy). In 87% of cases lesion of the supra aortic trunk, 29% with aortic lesions and renal arteries, 7.93% with isolated renal arteries and 7% with aortic coarctation cases, valvular heart disease in 7% of cases. Association with tuberculosis in 7.93% of cases and a dysthyroidism in 11.1% of cases. That 49% benefited from surgical treatment. Discussion: Involvement of the aorta and renal arteries seen in half of patients with TD is the leading cause of hypertension. In general, hypertension is severe, not or poorly controlled by medical treatment and is accompanied by cardiac and renal repercussions. Conclusion: Hypertension is common during TD; it is a poor prognostic factor, more serious than the cause is renovascular. Early diagnosis and appropriate therapy will help to prevent a handicap, which is sometimes major for these patients, most of whom are young. Surgical treatment of renal and aortic lesions of TD is indicated when there is severe hypertension, not or poorly controlled by heavy medical treatment, and the indications should be weighed well. The spontaneous evolution in these patients is burdened with a not insignificant mortality. Management and therapeutic decisions must be multidisciplinary.

Scleritis and Takayasu’s disease – rare combined presentation

Introduction: Takayasu’s disease (TD) (pulseless disease, aortic arch syndrome) is a rare but potentially life-threatening chronic giant cell vasculitis. The major complications attributed to the disease include Takayasu’s retinopathy, secondary hypertension, aortic regurgitation, and arterial aneurysm. The aim of this study is to describe a patient with Takayasu’s disease and scleritis and to show its association.Case report: We describe a 44-year-old female patient with Takayasu’s disease who presented with bilateral anterior scleritis in the form of painful red eye. Patient did not have any associated autoimmune diseases. Patient was diagnosed to have TD and was treated with immunosuppressant.Conclusion: Scleritis though rare can be a vasculitic feature of TD. This is a first report of Takayasu’s disease with scleritis, which has been documented in Nepal.