1/9. Choroidal vascular occlusion in a child with a connective tissue disease and complement c4 deficiency.OBJECTIVE: To report the histopathologic findings in the eyes of a patient with a connective tissue disease and complement deficiency. DESIGN: Human postmortem ocular histopathologic study. INTERVENTION: A 15-year-old female died from complications of a connective tissue disease of uncertain etiology, particularly acute respiratory distress syndrome. Abnormalities seen in the eyes at autopsy were consistent with complement activation, granulocyte aggregation, and leukocyte embolization. MAIN OUTCOME MEASURES: Both eyes were examined by light microscopy. RESULTS: Some choroidal vessels were occluded by platelet-fibrin thrombi and occasionally by aggregates of granulocytes and fibrin. Serous retinal detachment involving the macula and peripheral retina was present in both eyes. CONCLUSIONS: This is a report of the ocular histopathologic findings in a patient with connective tissue disease and complement c4 deficiency. The light microscopy findings were consistent with complement activation with granulocyte aggregation and leukocyte embolization and may represent another mechanism to explain the clinical findings in patients with connective tissue disease, particularly systemic lupus erythematosus.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. A case suggesting lymphocytic vasculitis as a presenting sign of early undifferentiated connective tissue disease.A previously healthy 14-year-old Korean male presented with striking hemorrhagic acral livedo associated with anti-ribonucleoprotein antibodies. His skin biopsy revealed a striking lymphocytic vascular reaction with mild superficial perivascular lymphocytic infiltrates, perivascular extravasation of red blood cells, and leukocytoclastic debris. Lymphocytic vasculitis (LV) is defined in different ways by different authors. The above biopsy findings do not fulfill the criteria for LV of all authors. Chronic lymphocyte-mediated endothelial cell injury may play an important role in the pathogenesis of collagen vascular disease. Cell-mediated cytotoxicity against endothelial cells or other vessel wall components is thought to be the possible pathologic mechanism of LV. We present a patient with early undifferentiated connective tissue disease (EUCTD) with hemorrhagic acral livedo, who finally developed systemic lupus erythematosus. Even though the histopathologic findings do not meet the most rigorous definition of LV, LV may a presenting sign of EUCTD. The purpose of this report is to suggest reconsideration of the diagnostic criteria of lymphocytic vasculitis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/9. Nonmyeloablative allogeneic bone marrow transplantation for treatment of childhood overlap syndrome and small vessel vasculitis.A 13-year-old Caucasian female with a systemic connective tissue disease (overlap syndrome with pulmonary vasculitis) underwent nonmyeloablative allogeneic BMT after failure of prolonged combination immunosuppressives to induce remission. The procedure also included cotransplantation of donor bone chips as a source of stromal cells. The unique protocol allowed good engraftment of hematopoietic (>95%) and bone core stromal cells (>60%). The patient was clinically improved, stable, and off all immunosuppressive medications 36 months post-transplant. To our knowledge, this is the first pediatric nonmyeloablative BMT with cotransplantation of stromal cells solely for treatment of an autoimmune disease.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
4/9. Pulmonary hypertension in connective tissue disease. Report of three cases and review of the literature.patients with connective tissue disease (CTD) who are prone to developed isolated pulmonary hypertension (PH) are primarily young females with a history of Raynaud's phenomenon associated with an exertional dyspnoea. From the start of the disease, pulmonary function tests show a decreased diffusing capacity for carbon monoxide, while X-ray examination shows no obvious abnormalities such as interstitial fibrosis. All patients show, on electrocardiographic examination, evidence of right axis deviation and right ventricular hypertrophy. It has been suggested that PH is found mostly in patients with systemic scerlosis characterized by the crest syndrome. The histopathological findings are intimal proliferation, narrowing of the vessel lumen and medial fibrosis. These are not specific for CTD. One would expect more signs of vasculitis. Neither signs of lung fibrosis, nor signs of pulmonary emboli are described.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/9. Necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC), and minimal cellular infiltration.Three adult patients, two with undifferentiated connective tissue disease and one with carcinoma, had a distinctive pathologic reaction pattern consisting of necrotizing myopathy, minimal cellular infiltration, and a microangiopathy with thick "pipestem" vessels and microvascular deposits of complement membrane attack complex. Quantitative analysis revealed focal capillary depletion. This pattern represents an immune-mediated microangiopathy and is distinct from that observed in other inflammatory myopathies.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/9. Unusual case of diffuse connective tissue disease with nodule formations in muscle, lung, kidney and brain.A 9-year-old boy had nodular masses in the left gastrocnemius muscle and lung, right kidney and right frontal area of the brain. Laboratory examinations, including positive anti-nuclear antibody, anti-RNP antibody, RA and CRP, hyper-gamma-globulinemia and accelerated ESR, showed characteristic of diffuse connective tissue disease (DCTD). biopsy specimens were obtained from the thymus and from masses in the left lung and gastrocnemius muscle. The thymus showed hyperplasia, and a mass in the lung showed nonspecific inflammatory reactions such as connective tissue proliferation and cellular infiltration. Biopsied muscle also showed severe connective tissue proliferation, cellular infiltration, variation in fiber size and thickened blood vessel walls. In addition, abnormalities, including thickening of the basement membrane and an almost occluded narrow lumen of capillaries, were found on electron microscopy examination. Steroid administration led to remarkable improvement of the symptoms. These results suggested that DCTD is responsible for these symptoms.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/9. Infantile systemic hyalinosis in a black infant.A black girl was born with flexion contractures and experienced pain on movement by 1 week of age. She subsequently developed perioral papules, gingival hyperplasia, perianal nodules, torticollis, diarrhea, rectal prolapse, and inability to open her mouth. Her skin became increasingly sclerodermatous, and velvety, hyperpigmented plaques arose over bony prominences. A skin biopsy specimen showed hyaline material in the papillary dermis with lack of elastic fibers. Ultrastructural examination revealed fibrillogranular material around fibroblasts and blood vessels. This child had the clinical, histologic, and ultrastructural features of infantile systemic hyalinosis. This disorder has not been described in a black infant. Previous case reports of infantile systemic hyalinosis are reviewed and unusual features of our case are discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/9. Multiple arteriovenous malformations in a man with a connective tissue disorder.Multiple arteriovenous malformations in patients with connective tissue disorders are not reported. We describe a man with cutaneous, ligament/articular and vascular features suggestive of a connective tissue disorder and who also has multiple arteriovenous malformations resulting in a high output circulatory state. chest pain due to arteriovenous malformations originating from subclavian vessels has caused considerable morbidity.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/9. Systemic sclerosis terminating as systemic necrotizing angiitis.We report a 57-year-old woman with systemic sclerosis (SSc) who died suddenly, following a haemoptysis. At post-mortem, systemic necrotizing angiitis of small vessels was observed in several organs. Necrotizing angiitis has been reported as a rare complication of systemic sclerosis, and is usually lethal.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |