1/26. Reversible pulmonary hypertension in poems syndrome--another etiology of triggered pulmonary vasculopathy?Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome, a plasma cell dyscrasia associated with pulmonary hypertension, has been treated in the past with anticytokine strategies with a poor outcome. A patient is described who had poems syndrome with precapillary pulmonary hypertension and Raynaud's phenomenon, and who responded well to a short course of corticosteroids and long term nifedipine. poems syndrome, like anorexigens, cocaine, portal hypertension and human immunodeficiency virus infection, may be another trigger for the pulmonary vascular occlusive process found in primary pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/26. Raynaud's phenomenon, anticentromere antibodies, and digital necrosis without sclerodactyly: an entity independent of scleroderma?We describe 4 women of 43, 73, 84, and 86 years with Raynaud's phenomenon, severe digital necrosis, and high serum levels of anticentromere antibodies without skin thickening or internal organ sclerosis. Investigations revealed no diabetes or arterial vascular disease leading to arterial obstruction. Histologic examination did not show any dermal sclerosis or calcinosis. The intravenous infusions of prostaglandin reversed the ischemic lesions in 3 patients. These cases suggest that the triad Raynaud's phenomenon, anticentromere antibodies and necrosis of digits without sclerodactyly and sclerosis of internal organ should be considered as an entity distinct from scleroderma with sclerosis. For this entity we propose the name RACAND syndrome.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
3/26. Idiopathic myelofibrosis associated with classic polyarteritis nodosa.A woman with scleroderma and classic polyarteritis nodosa (PAN) who developed idiopathic myelofibrosis (IM) is reported. The patient presented with a one-year history of weakness, polyarthritis, Raynaud phenomenon, dry cough, and epigastralgia. The diagnosis of scleroderma with visceral involvement was made and treatment with prednisone subsequently started, with good clinical response. Six years later, fever, weight loss, livedo reticularis, and dysesthesias developed. Electromyographic studies were consistent with sensory neuropathy and a sural nerve biopsy yielded the diagnosis of PAN. The patient received cyclophosphamide plus prednisone with a favorable response, but 11 years later she was admitted because of weakness, constitutional symptoms, and abdominal pain due to spleen infarcts. Marked anemia, with aniso-poikilocytosis, tear-drop cells, immature myeloid precursors in the peripheral blood, and an increased serum LDH, was observed and the diagnosis of IM established by bone marrow biopsy. This case represents a new association between IM and an autoimmune disease and supports the hypothesis of an immune basis of IM in some patients.- - - - - - - - - - ranking = 8.9562576589056keywords = nerve (Clic here for more details about this article) |
4/26. 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 = 1keywords = organ (Clic here for more details about this article) |
5/26. Primary sjogren's syndrome presenting with generalized autonomic failure.A 64 year-old woman developed Raynaud's phenomenon and dry eyes/mouth. Laboratory examination revealed positive Schirmer's test, rheumatoid factor and anti-nuclear antibody, and lymphocytic sialoadenitis on salivary gland biopsy. These features strongly suggested the diagnosis of primary sjogren's syndrome. Three years later, she gradually developed generalized autonomic failure without apparent sensory neuropathy on nerve conduction study. She had systolic pressure fall of 51 mmHg on head-up tilt test, cardiovascular supersensitivity to diluted norepinephrine infusion, cardiac denervation in [123I]-MIBG scintigraphy, impaired R-R variability, decreased sweating and prolonged colonic transit time. Autoimmune autonomic ganglionopathy was mostly responsible for her autonomic failure.- - - - - - - - - - ranking = 8.9562576589056keywords = nerve (Clic here for more details about this article) |
6/26. Multiple mononeuropathy due to vasculitis associated with anticardiolipin antibodies: a case report.This report illustrates a case of peripheral nerve vasculitis associated with elevated anticardiolipin antibodies. A 49-year-old female with a history of seven spontaneous abortions initially complained of pain and numbness in her right calf that later spread to the left foot and ankle. Over the next few months, she developed a Raynaud phenomenon and livedo reticularis. Clinical examination revealed signs of multiple mononeuropathy. Right sural nerve biopsy performed two months after the beginning of the disease revealed active necrotizing arteritis of the epineural arteries with transmural inflammatory infiltrate and thrombosis. Vasculitis is a rare finding in sural nerve biopsies, usually in patients with systemic vasculitis or autoimmune connective tissue diseases. However, vasculitis restricted to the peripheral nerves has also been described. Our patient had no clinical or laboratory features of any autoimmune disorder and also no signs of systemic vasculitis. We discuss the potential role of anticardiolipin antibodies in the pathogenesis of vasculitis.- - - - - - - - - - ranking = 35.825030635623keywords = nerve (Clic here for more details about this article) |
7/26. Peripheral neuropathy in monoclonal gammapathy with cryoglobulinemia and arteritis.We report a patient with IgM gammapathy, cryoglobulinemia, Raynaud's phenomena, purpura hyperglobulinemica of the legs and polyneuropathy. Endoneural vasculitis with infiltrations of eosinophilic and neutrophilic granulocytes and an extensive loss or wallerian degeneration of myelinated nerve fibers were seen on histopathologic examination of a sural nerve biopsy specimen. The microscopic picture differed somewhat from that observed previously in cryoglobulinemic vasculitis. Although vasculitis is most often believed to represent an immunologically mediated lesion, we propose an alternate explanation, namely, that the disease manifestations in the present case were secondary to cold-induced effects of the cryoglobulin on the microcirculation.- - - - - - - - - - ranking = 17.912515317811keywords = nerve (Clic here for more details about this article) |
8/26. Organ specific and multisystemic autoimmune disease: part of a spectrum which may coexist in the same patient.Five patients are described who were found to have both systemic lupus erythematosus and autoimmune thyroid disease. The coexistence of nonorgan specific and organ specific autoimmune disease is discussed.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
9/26. Peripheral nerve injury and Raynaud's syndrome following electric shock.A truck driver was injured by a high-voltage line of 10,000 volts when holding a metallic bar in both hands. Initially no neurological abnormalities were found, but during the following few weeks increasing sensory and minor motor symptoms developed in the right upper extremity. After one year numbness of the right thigh and leg appeared, as well as attacks of white finger in both hands. Repeated examinations showed progressive abnormalities of the median and ulnar nerves in both hands. No other cause for Raynaud's syndrome was discovered. The late high-voltage effects, presumably indirect, are suggested to be of multifactorial etiology.- - - - - - - - - - ranking = 44.781288294528keywords = nerve (Clic here for more details about this article) |
10/26. Ultrastructural changes in peripheral nerves of the fingers of three vibration-exposed persons with Raynaud's phenomenon.A finger biopsy was performed on three patients with vibration-induced white finger (VWF), and the specimens were examined by electron microscopy for peripheral nerve changes. A characteristic neuropathy with perineurial fibrosis was revealed which was often accompanied by a thickened perineurium with a lamellar structure resembling onion skin. This peculiar fibrosis consisted of elongated cytoplasmic projections of perineurial cells or fibroblasts and a greatly increased amount of collagen which occasionally contained fibrous long-spacing collagen. In the endoneurium, a decrease in the number of nerve fibers and a marked increase in collagen with fibroblasts were noted. Myelinated axons became smaller, and this occurrence suggested incomplete regeneration. The pathological changes were presumably the result of the long-term clinical course of VWF.- - - - - - - - - - ranking = 53.737545953434keywords = nerve (Clic here for more details about this article) |
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