1/22. Beware of the heart: the multiple picture of cardiac involvement in myositis.A 42-yr-old woman with dermatomyositis had two myocardial infarctions, episodes of acute chest pain and an acute lung oedema. These events were initially misinterpreted as atherosclerotic ischaemic heart disease accompanying the autoimmune disease. The lack of improvement of cardiac symptoms with anti-ischaemic and immunosuppressive drugs indicated other mechanisms. Intracoronary drug provocation as well as myocardial biopsy revealed a coincidence of small-vessel disease and vasospastic angina as a cause for the severe cardiac symptoms. After initiating therapy with high doses of calcium channel blockers, marked improvement of cardiac symptoms occurred. In the pathogenesis of cardiac involvement in dermatomyositis, two different mechanisms should be considered: inflammatory processes due to dermatomyositis and vasoconstriction caused by an impaired regulation of vascular tone, such as abnormal vessel reactivity or disturbed neuropeptide release. Signs of this generalized vasopathy are Raynaud's phenomenon, Prinzmetal's angina and small-vessel disease, which can coincide. In patients with severe cardiac symptoms and autoimmune diseases, Prinzmetal's angina should be excluded by intracoronary drug provocation using acetylcholine.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/22. Raynaud's phenomenon possibly induced by a compund drug of tegafur and uracil.We describe a 50-year-old woman who noted acral hyperpigmentation, sclerodactyly and Raynaud's phenomenon with 1:160 of antinuclear antibody titer after treatment with a compound drug with tegafur and uracil. Histological findings of the finger and palm included hyperkeratosis, vacuolar degeneration of basal cells, thickened collagen fibers in the dermis, and dilatation of capillary vessels, perivascular mononuclear cell infiltration and melanophages in the upper dermis. IgG, IgA, IgM, C3 and C1q were not deposited in the skin by direct immunofluorescence study. After cessation of the drug, Raynaud's phenomenon and hyperpigmentation disappeared within 1 month and 4 months, respectively, and antinuclear antibody turned negative within 4 months. These observations suggest that tegafur may have caused not only hyperpigmentation in the palms and soles, but also sclerodactyly and Raynaud's phenomenon in the present case.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
3/22. thromboangiitis obliterans: a rare cause of a reversible Raynaud's phenomenon.A 25-year-old woman with progressive Raynaud's phenomenon and digital necrosis is presented. Systemic sclerosis and other connective tissue disorders as well as atherosclerosis and arterial emboli were excluded with appropriate laboratory examinations. Arteriography revealed multiple palmar and digital occlusions with corkscrew-shaped vessels. Based on these characteristic arteriographic and clinical findings, the diagnosis of thromboangiitis obliterans was finally retained. With intravenous perfusion of the prostacyclin analogue iloprost (2 ng/kg/min, 6 h daily during 21 days), a complete healing of Raynaud's phenomenon and of the digital necrosis was observed. There was no recurrence during the 1-year follow-up. This observation demonstrates that thromboangiitis obliterans is a potential reversible cause of severe Raynaud's phenomenon in young women even in the absence of lower limb involvement. Early recognition is important to avoid irreversible complications such as loss of digits.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
4/22. hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud's phenomenon complicated by digital gangrene.Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES.- - - - - - - - - - ranking = 0.66666666666667keywords = vessel (Clic here for more details about this article) |
5/22. Migraine and Raynaud phenomenon: possible late complications of Kawasaki disease.Migraine and Raynaud phenomenon often coexist and may reflect similar vascular reactions. Both have been associated with vascular endothelial cell dysfunction. Kawasaki disease is a systemic vasculitis of unknown etiology that affects children and may lead to the formation of coronary artery aneurysms. Endothelial cell dysfunction has been demonstrated late in Kawasaki disease and is not restricted to coronary vessels. We report the case of a patient who developed typical migraine with aura and Raynaud phenomenon at the age of 14, 12 years after onset of Kawasaki disease. His migraine responded well to pizotifen, and both migraine and Raynaud phenomenon improved after initiation of treatment with valproic acid. We postulate that both migraine and Raynaud phenomenon in this case represent late consequences of Kawasaki disease and result from extracoronary endothelial dysfunction.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
6/22. The many faces of scleroderma.This review integrates the clinical aspects of systemic sclerosis (SSc; scleroderma) and scleroderma-like conditions with new knowledge of the control of blood vessel tone and the role of anoxia in the activation of connective tissues leading to fibrosis. serologic tests, high resolution computed tomographic scanning, bronchoalveolar lavage, and physiologic assessment of pulmonary gas diffusion are compared as diagnostic tools and as means of quantitating internal organ involvement. Treatment of Raynaud's disease and phenomenon, management of scleroderma renal crisis, and new means for improving gastrointestinal function with octreotide, the somatostatin analogue, also are discussed. The relationship between idiopathic forms of SSc and eosinophilic fasciitis/eosinophilia-myalgia syndrome caused by L-tryptophan ingestion and the scleroderma-like disease associated with silicone breast implants also is discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
7/22. Capillary occlusion and secondary angiogenesis: case report.The first follow-up control 5 days later (14.1.1990) showed a fresh capillary occlusion with massive red blood cell diapedesis. The arteriolar limb of the capillary is filled with erythrocytes up to the occlusion. Since capillary occlusion probably allowed some plasma flow at first, blood cells distal to the occlusion were washed away. After 53 days (8.3.1990) extravasal cells could no longer be made out. At the upper margin of the image a newly formed capillary loop can be seen. The channel of the old capillary seemed to have disappeared. Seventy-three days after occlusion, on 28th of March, the tip of the capillary, already demonstrated on 8th of March, had further grown distally. The capillary had crossed the neighbouring diagonal capillary. Twelve days later (9.4.1990) a remarkable growth connected with formation of a collateral was observed. The new capillary grew in direction of the former capillary channel. On 22nd of April the new capillary had almost got back to its old form. The collateral vessel formed on 9th of April had also grown and could be well distinguished. The different branches were immediately well perfused (mean erythrocyte velocity is v = 0.74 mm/s). The capillary were growing at different speeds. The new capillary had visibly grown by 220 microns over a period of 47 days which corresponds to an average growth of 4.7 microns a day. The capillary was growing fastest between the 9th and 22nd of April. A minimum of growth of 2 microns was observed within the first 11 days of the observation period.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
8/22. Treprostinil for the treatment of severe digital necrosis in systemic sclerosis.We report a case of severe digital ulcerations associated with systemic sclerosis, successfully treated with treprostinil (Remodulin). There was improvement within days of the treatment initiation; complete healing was accomplished after 16 weeks of therapy. patients with systemic sclerosis and peripheral small vessel disease have limited therapeutic options. Treprostinil is a prostacyclin analogue that can be delivered by subcutaneous infusion and is approved in the USA only for treatment of primary pulmonary hypertension. This report provides an impressive example of an alternative, complementary indication for the use of treprostinil.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
9/22. Decreased rectal body temperature induced by different vasodilatory drugs.In three patients with either hypertension or Raynaud's phenomenon who were treated with different types of vasodilatory drugs, i.e. prazosin, nifedipine and prostacyclin, a decrease in rectal body temperature was measured. Overt hypothermia occurred in one patient. Because we found a pronounced improvement of arteriovenous shunt flow within the skin vessels in two of these patients, increased heat loss seems the most reasonable explanation for the observed decrease in body temperature. These observations point to an adverse effect of vasodilatory drugs. One should be aware of this possibility in patients being treated with these drugs who have complaints suggestive of hypothermia.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
10/22. The clinical application of forearm and hand blood flow measurements using a gamma camera.A new method of measuring blood flow to forearms and hands during reactive hyperemia is described. Subjects are positioned with forearms and hands over a gamma camera, and the circulation below the elbows is isolated for 4 minutes with pneumatic cuffs. The remaining blood pool is labeled with technetium-99m-labeled human serum albumin given via a vein in the dorsum of the foot. The rate of rise of activity in the arms after cuff release is measured from the gradient of time activity curves, and these are calibrated for flow by counting a sample of venous blood. Blood flow per unit volume is obtained from measurements of the limb volume, done by water displacement. Results for eight normal control subjects showed a mean flow per unit volume to forearm plus hand of 32.9 /- 6.4 ml/100 ml/min and to hand alone 36.5 /- 6.9 ml/100 ml/min. Case studies of patients with a variety of large- and small-vessel diseases are presented, illustrating the use of the technique to aid diagnosis and measure response to treatment.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
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