1/39. mixed connective tissue disease.Three patients with mixed connective tissue disease (MCTD) had clinical features that included a high incidence of Raynaud phenomenon, arthritis, myositis, and swollen hands. The diagnostic laboratory test result was the presence of high titers of antibody to extractable nuclear antigen. These antibody titers are notably reduced or abolished in patients with MCTD when the tanned red blood cells that are used in the test are pretreated with ribonuclease. Speckled antinuclear antibodies were present in all patients. patients with MCTD have a low incidence of renal disease, are responsive to treatment with prednisone, and have a good prognosis.- - - - - - - - - - ranking = 1keywords = mixed connective, connective (Clic here for more details about this article) |
2/39. 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 = 0.055682605620234keywords = connective (Clic here for more details about this article) |
3/39. 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 = 0.055682605620234keywords = connective (Clic here for more details about this article) |
4/39. mixed connective tissue disease associated with von Recklinghausen's neurofibromatosis.We report a 42-year-old Japanese woman with Recklinghausen's neurofibromatosis 1 (NF1) who developed mixed connective tissue disease (MCTD). Previously experiencing good health without an increase in subcutaneous nodules, she presented with Raynaud's phenomenon, swollen hands and polyarthralgia Clinical examination revealed a high titer of anti-RNP antibody, and she was thus diagnosed as having MCTD. She was treated with oral prednisolone (10 mg/day) and her symptoms improved rapidly. Since the association of MCTD and NF1 has not been reported previously, we concluded that this association is rare. We also discussed the association of NF1 and autoimmune diseases including MCTD.- - - - - - - - - - ranking = 1keywords = mixed connective, connective (Clic here for more details about this article) |
5/39. Efficacy of pulsed intravenous immunoglobulin therapy in mixed connective tissue disease.We describe a 69-year-old patient with long-standing mixed connective tissue disease who suffered from severe skin eruptions that did not respond to various immunosuppressive regimens. Therapy with high-dose intravenous immunoglobulin was successful in controlling the patient's disease without major side effects. We think that this regimen-although expensive-might be an interesting therapeutic option in selected patients with mixed connective tissue disease that is refractory to other treatment modalities.- - - - - - - - - - ranking = 4.6636174651144keywords = mixed connective, connective (Clic here for more details about this article) |
6/39. Transdermal application of prostaglandin E1 ethyl ester for the treatment of trophic acral skin lesions in a patient with systemic scleroderma.An early dinical symptom in scleroderma patients is Raynaud's phenomenon. Later cutaneous manifestations of the disease include oedematous swelling in the extremities and in more extreme cases often very painful, refractory acral necroses. We report on a 56-year-old female patient who participated in a prospective, double-blind, multicentre comparative pilot study because of her severe Raynaud symptoms, with dystrophic skin lesions on both hands. The goal of the study was to evaluate the efficacy and safety of prostaglandin E1 ethyl ester in a transdermal drug delivery system compared with placebo in patients with secondary Raynaud's phenomenon associated with systemic scleroderma or mixed connective tissue disease. After 2 weeks of verum treatment the patient experienced a marked improvement of Raynaud's attacks, with increased capillary flow velocity, reduced blood stasis and dinical healing of the acral trophic lesions. For this patient the transdermal application of prostaglandin E1 ethyl ester in the form of a medicated patch proved to be a simple and effective therapy for the acral trophic skin lesions associated with systemic scleroderma.- - - - - - - - - - ranking = 0.77726957751906keywords = mixed connective, connective (Clic here for more details about this article) |
7/39. Continuous regional anesthesia before surgical peripheral sympathectomy in a patient with severe digital necrosis associated with Raynaud's phenomenon and scleroderma.BACKGROUND AND OBJECTIVE: Digital ischemia and necrosis caused by Raynaud's phenomenon in patients with connective tissue diseases may not respond to medical therapy and may have major adverse effects on quality of life. We describe the use of continuous ambulatory regional anesthesia for diagnosis and treatment before peripheral sympathectomy in a patient with secondary Raynaud's phenomenon. CASE REPORT: A 55-year-old man with progressive systemic sclerosis and secondary Raynaud's phenomenon presented with severe pain and digital necrosis that were refractory to maximal medical treatment and thoracic sympathectomy. Continuous ambulatory regional analgesia increased digital temperature from 32.3 degrees C at baseline to 34.4 degrees C after 80 minutes. An increase in digital flow was documented by Doppler ultrasound measurements made ventrally at the point of greatest pulsation of the radial artery. Subsequent peripheral sympathectomy resulted in restoration of nutrient flow with healing of ulcers and alleviation of pain. CONCLUSIONS: Continuous ambulatory regional anesthesia appears effective as a treatment bridge for vasospasm and ischemia associated with secondary Raynaud's phenomenon. The enhancement of peripheral blood flow achieved with the regional anesthetic technique suggests that surgical peripheral sympathectomy may provide long-term benefits.- - - - - - - - - - ranking = 0.055682605620234keywords = connective (Clic here for more details about this article) |
8/39. 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 = 0.055682605620234keywords = connective (Clic here for more details about this article) |
9/39. A case of Raynaud's disease with uterine cancer producing interleukin-6.A case of cervical cancer of the uterine producing interleukin-6(IL-6) in a patient who suffered from Raynaud's phenomenon is described. Her serum contained anti SS-A antibody. The cancer was removed surgically. High level of IL-6 activity was detected in the culture supernatant of the resected cancer cells. After operation, Raynaud's phenomenon had improved and anti SS-A antibody had disappeared. This case shows us that IL-6 produced by malignant tumour might induce autoimmune connective tissue disease-like symptoms.- - - - - - - - - - ranking = 0.055682605620234keywords = connective (Clic here for more details about this article) |
10/39. erythema elevatum diutinum in the setting of connective tissue disease and chronic bacterial infection.erythema elevatum diutinum (EED) is a rare and chronic cutaneous leukocytoclastic vasculitis. It is predominantly seen on the extensor surfaces of the extremities. Although a specific cause is largely unknown, EED has been noted to occur in association with a wide variety of diseases. A 28-year-old man with systemic lupus erythematosus (SLE) and a 53-year-old woman with an overlap syndrome of rheumatoid arthritis and polymyositis are presented. Both patients developed EED in the setting of chronic recurrent bacterial infections. patients with a connective tissue disease are at increased risk for such infections secondary to immunosuppression, either from the disease itself or secondary to immunosuppressive therapy. EED has been independently reported to occur in the setting of connective tissue disease as well as in the setting of chronic infection. Our patients had both of these underlying conditions, which are known to predispose patients to immune complex-mediated vasculitides, in this case EED. One patient's EED responded to treatment of the SLE and the other improved, as has been previously reported with dapsone.- - - - - - - - - - ranking = 0.33409563372141keywords = connective (Clic here for more details about this article) |
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