1/16. Phlebosclerosis of the colon with positive anti-centromere antibody.A 56-year-old woman with symptoms of chronic bowel disease presented a peculiar calcification of the mesenteric vein of the ascending to transverse colon on barium enema study. The resected colon was hard and black. Histo-pathologic examinations demonstrated fibrous change of the colon with a calcified and hyaline-deposited mesenteric vein. No cell infiltration was observed. These findings were compatible with phlebosclerosis and also with systemic sclerosis. Positive anti-centromere antibody and Raynaud's phenomenon, hallmarks of a variant systemic sclerosis, the crest syndrome were observed. We therefore speculated that the pathogenesis of the phlebosclerosis of the colon is related to the crest syndrome.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/16. A case of crest syndrome and myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis.We report the first case of myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA)-associated glomerulonephritis in a patient with crest syndrome. A 74-year-old Japanese man with crest syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) developed rapidly progressive renal failure without elevation of blood pressure. Renal biopsy revealed glomerular sclerosis and fibrous crescents. The MPO-ANCA titer was elevated to 145 EU/ml. When patients with collagen diseases develop rapidly progressive glomerulonephritis, the possibility of MPO-ANCA-associated glomerulonephritis should be kept in mind.- - - - - - - - - - ranking = 0.125keywords = sclerosis (Clic here for more details about this article) |
3/16. autonomic nervous system and smooth muscle cell involvement in systemic sclerosis: ultrastructural study of 3 cases.OBJECTIVE: To investigate morphological abnormalities in nerve and smooth muscle structures of the anorectal wall underlying gastrointestinal dysfunction in patients with systemic sclerosis (SSc). methods: We performed deep rectal biopsy in 3 patients with limited scleroderma of relatively recent onset and intestinal symptoms. RESULTS: We found ultrastructural signs of axonal degeneration and cytoskeletal abnormalities in the bundles of unmyelinated fibers. There was also focal degeneration of smooth muscle cells, often in association with the presence of partially degranulated mast cells. Many mast cells were also observed in close relation to nerve fibers and vessels. The enteric vessels often showed basal lamina reduplication and hypertrophied endothelial cells with obliterated lumen. No significant fibrosis was found. CONCLUSION: Our findings indicate early involvement of the autonomic nervous system and to a lesser extent of smooth muscle cells. We confirmed the presence of early vascular lesions and involvement of mast cells in the pathological process.- - - - - - - - - - ranking = 0.625keywords = sclerosis (Clic here for more details about this article) |
4/16. Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review.Systemic sclerosis is a multi-system disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Although resorption of the mandible has been commonly observed and reported, we found no report of resorption leading to a painful neuropathy of the inferior alveolar nerve. We report a case of a patient with systemic sclerosis, severe resorption of the angles, and inferior alveolar border of the mandible, resulting in a compression neuropathy of the inferior alveolar nerve. Diagnostic tests, medical treatment, and surgical treatment are discussed, and the relevant literature is reviewed.- - - - - - - - - - ranking = 0.75keywords = sclerosis (Clic here for more details about this article) |
5/16. Granulomatous anterior uveitis in a patient with crest syndrome.The crest syndrome is a variant form of progressive systemic sclerosis. Apart from the occurrence of keratoconjunctivitis sicca, other types of ocular involvement associated with this variant are quite rare. We present the case of a 73-year-old woman with the CREST variant of progressive systemic sclerosis who developed unilateral granulomatous anterior uveitis. Systemic and laboratory testing failed to suggest evidence for any other associated systemic disease as a possible cause of the granulomatous uveitis. The inflammation was successfully controlled with topical steroids and mydriatics. While a small number of cases of uveitis have been reported in other variant forms of progressive systemic sclerosis, to date there have been no descriptions of uveitis associated with the crest syndrome.- - - - - - - - - - ranking = 0.375keywords = sclerosis (Clic here for more details about this article) |
6/16. Autoimmune hepatitis and systemic sclerosis: a new overlap syndrome?OBJECTIVE: We report the cases of two patients with the complete CREST variant (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) of systemic sclerosis (SSc) who developed autoimmune hepatitis. RESULTS: Our findings suggest that autoimmune hepatitis can be considered to be one of the liver manifestations associated with SSc. Our data also indicate that, because liver involvement may precede skin manifestations, evaluation for SSc is appropriate when autoimmune hepatitis is noted, and that the evaluation should include clinical examination, testing for antinuclear antibodies (especially for anticentromere antibodies) and nailfold capillaroscopy. CONCLUSIONS: From a practical point of view, our two cases emphasize that suspicion of autoimmune hepatitis in SSc patients presenting with cytolytic hepatitis will help to achieve both accurate diagnosis and optimal management.- - - - - - - - - - ranking = 0.625keywords = sclerosis (Clic here for more details about this article) |
7/16. Systemic sclerosis sine scleroderma: is it always the same disease? Report of three patients and discussion.The recent description of a large cohort of patients with the diagnosis of systemic sclerosis sine scleroderma (ssSSc) provided significant progress in our understanding of this entity. The prognosis of patients with ssSSc is, however, very variable, from benign in most cases to rapidly disabling in others. By reporting three new cases and analyzing previously published data, we discuss possible subsets and variants of the disease form.- - - - - - - - - - ranking = 0.625keywords = sclerosis (Clic here for more details about this article) |
8/16. bronchiectasis in a patient with crest syndrome.bronchiectasis is an uncommon pulmonary manifestation of systemic sclerosis (SSc). We report the case of a 70-year-old woman with crest syndrome and vasculitis who developed multifocal symptomatic bronchiectasis. The bronchiectasis and immunosuppressive therapy precipitated severe lower respiratory tract infection, which was fatal within a few months. The concomitant occurrence of bronchiectasis and SSc raises the possibility of a pathophysiological relationship. Several hypotheses can be put forward to explain the occurrence of bronchial wall damage leading to bronchiectasis. Whatever the mechanism, cases of bronchiectasis in patients with SSc should be reported to make physicians aware of the substantial risk associated with this combination.- - - - - - - - - - ranking = 0.125keywords = sclerosis (Clic here for more details about this article) |
9/16. Cranial nerve VIII involvement in a patient with progressive systemic sclerosis.Systemic sclerosis is a multisystem disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Cranial nerve involvement is an uncommon feature of this connective tissue disorder, and when it occurs it is the trigeminal nerve that is primarily affected. We report an elderly woman who presented with sensorineural hearing loss and was then diagnosed with the crest syndrome of progressive systemic sclerosis (calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia). Involvement of the eighth cranial nerve with scleroderma and crest syndrome is rare, but appears to be the cause of sensorineural hearing loss in our patient.- - - - - - - - - - ranking = 0.75keywords = sclerosis (Clic here for more details about this article) |
10/16. Solitary extramedullary plasmacytoma and granulomatous sialadenitis of the parotid gland preceding a B-cell non-Hodgkin's lymphoma.A patient with swelling of the left parotid gland of four-months' duration, sicca syndrome (xerophthalmia and xerostomia) and a history of progressive systemic sclerosis with an incomplete form of the crest syndrome was referred to our department. On ultrasound a parotid mass of reduced echogenicity without any enlarged cervical lymph nodes was found. Ultrasonographically guided fine-needle biopsy could not provide any definitive diagnosis. After partial parotidectomy with complete tumor removal the histologic exam showed an extramedullary plasmacytoma with concurrent non-necrotizing granulomatous sialadenitis of the parotid gland. Complete systemic work-up excluded multiple myeloma, leukemia, lymphoma and sarcoidosis. Post-operative radiotherapy of the left parotid region and left neck including the supraclavicular lymph node area was performed. Six months after surgery an aggressive B-cell non-Hodgkin's lymphoma was diagnosed.- - - - - - - - - - ranking = 0.125keywords = sclerosis (Clic here for more details about this article) |
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