1/14. Granulomatous pneumocystis carinii pneumonia in Wegener's granulomatosis.This study reports on a first case of granulomatous pneumocystis carinii pneumonia (PCP) in a human immunodeficiency virus-negative patient with antineutrophil cytoplasmic antibody-positive Wegener's granulomatosis whilst receiving immunosuppressive treatment. The patient presented with diffuse alveolar haemorrhage, pauci-immune rapid progressive glomerulonephritis and leukocytoclastic vasculitis of the skin. Granulomatous pneumocystis carinii pneumonia developed under immunosuppressive treatment with cyclophosphamide and prednisone. At the time pneumocystis carinii pneumonia developed, there was a marked lymphopenia with a very low CD8 cell count in the blood. Grocott staining in bronchoalveolar lavage fluid revealed no pneumocystis carinii. The diagnosis was made via a video-assisted thoracoscopic lung biopsy which showed granulomas containing high numbers of pneumocystis carinii cysts.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
2/14. Acute upper gastrointestinal haemorrhage and colitis: an unusual presentation of Wegener's granulomatosis.Wegener's granulomatosis is a rare necrotizing vasculitis usually affecting the respiratory tract and kidneys. The aetiology is unknown and it usually occurs in patients over the age of 40. Involvement of the gastrointestinal tract in Wegener's granulomatosis is relatively rare and usually occurs long after the onset of initial symptoms. Acute colitis as a presenting feature of Wegener's granulomatosis is very rare with only a few reports in the literature. We describe a young woman who presented initially to hospital with gastrointestinal features and then developed a severe colitis and severe gastrointestinal haemorrhage. This preceded the development of respiratory tract features with severe pulmonary haemorrhage, haemoptysis and the development of rapidly progressive renal failure and nasal septal perforation. Following treatment with intravenous steroids and cyclophosphamide, gastrointestinal symptoms and signs improved dramatically, as did her pulmonary disease. She still remains dialysis dependent, due to end-stage renal disease secondary to glomerulonephritis.- - - - - - - - - - ranking = 6keywords = haemorrhage (Clic here for more details about this article) |
3/14. Antineutrophil cytoplasmic antibodies (ANCA) and small vessel vasculitis.We report on one patient with Wegener's granulomatosis (WG) and two patients with microscopic polyangiitis (MPA). The patient with WG had signs of a respiratory infection and showed a c-ANCA pattern with proteinase 3 (PR3) specificity. The patients with MPA presented with pulmonary haemorrhage and signs of renal damage and showed a p-ANCA pattern with myeloperoxidase (MPO) specificity. In the three patients histopathological findings confirmed the diagnosis. We discuss the clinical indications of ANCA testing and the current terminology for reporting ANCA results (c-ANCA, p-ANCA, c-ANCA (atypical) and atypical ANCA). The target antigens and diseases associated with these different patterns are considered. Finally we focus on the value of ANCA and more specific PR3-ANCA and MPO-ANCA in the diagnosis of WG and MPA. The new application domain of ANCA in Crohn's disease and ulcerative colitis is also discussed.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
4/14. eosinophilia and hyperimmunoglobulinemia E as the presenting manifestations of Wegener's granulomatosis.eosinophilia has long been known as a hallmark of churg-strauss syndrome but has rarely been reported in Wegener's granulomatosis (WG). Here we describe a patient with WG who had skin, kidney and lung involvement as well as striking peripheral eosinophilia and hyperimmunoglobulinaemia E (hyper-IgE). The patient's clinical picture was complicated by intra-alveolar haemorrhage resulting in severe anaemia and respiratory failure. The pulmonary symptoms recovered completely, but the renal involvement evolved into end-stage renal failure despite intensive immunosuppressive treatment, intravenous immunoglobulin and plasmapheresis. We suggest that the presence of eosinophilia and hyper-IgE might contribute to the development of different disease patterns in WG.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
5/14. Small bowel haemorrhage due to cytomegalovirus vasculitis.A case is described of a life threatening vasculitis of the small bowel leading to massive gastrointestinal haemorrhage which was apparently due to cytomegalovirus inclusion disease. Reactivation of cytomegalovirus probably followed the treatment of Wegener's granulomatosis with corticosteroids and azathioprine. This patient was treated successfully by surgical excision of the affected segment of ileum together with intravenous ganciclovir.- - - - - - - - - - ranking = 5keywords = haemorrhage (Clic here for more details about this article) |
6/14. Diffuse granulomatous necrotizing scleritis.CASE REPORT: A 34-year-old man presented with anterior scleral thinning in the right eye (RE) and a painful nodular scleritis in the left eye (LE). Fundus examination showed a healed vasculitis and an inferior epiretinal neovascular membrane in the LE. Topical and systemic oral steroids and antiviral medication were prescribed. One year later, optic disc hyperaemia and swelling and macular oedema became apparent in the LE. Pulsed intravenous steroids were administered for 1 year, when a nasal septum perforation and vitreous haemorrhage in the LE were diagnosed. The eye was enucleated 3.5 years after the initial complaint. Necrotizing granulomatous tissue replacing the sclera and subconjunctival granulomatous tissue were observed. Six months later, oedema and neovascularization of the right optic disc were observed and cyclophosphamide was started, with regression of the clinical signs. No systemic abnormalities have so far become apparent. Discussion: Posterior scleritis is most often observed in patients with no signs of associated systemic autoimmune disease. The diagnosis in this case is most probably one of Wegener's granulomatosis (WG). In WG, the diagnosis is based on necrotizing granulomas of the respiratory tract, generalized focal necrotizing vasculitis and focal necrotizing glomerulonephritis. eye involvement with WG has been reported in up to 58% of cases. Evaluation of the scleritis patient should include a detailed medical history, chest x-rays, blood tests, autoantibody serology and analysis of urinary sediment. cyclophosphamide is the treatment of choice for patients with ocular manifestations of WG, polyarteritis nodosa or rheumatoid arthritis, either alone or in combination with systemic steroids. Visual loss is expected in 85% of individuals with severe necrotizing posterior scleritis.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
7/14. Subarachnoid haemorrhage in Wegener's granulomatosis, with negative four vessel angiography.Two patients with Wegener's granulomatosis and spontaneous subarachnoid haemorrhage are presented in whom four vessel angiograms were normal. The diagnosis of Wegener's granulomatosis should be considered in patients with subarachnoid haemorrhage and negative four vessel angiography. The presence of antibodies to a neutrophil cytoplasmic antigen may be of diagnostic value.- - - - - - - - - - ranking = 6keywords = haemorrhage (Clic here for more details about this article) |
8/14. Wegener's granulomatosis presenting as diffuse pulmonary hemorrhage.A 35-year-old woman experienced diffuse intraalveolar haemorrhage with respiratory distress and acute renal failure. Renal histology and evolution confirmed Wegener's granulomatosis. Early use of immunosuppressive drugs allowed weaning from mechanical ventilation and temporary improvement of the renal failure. A review of the literature emphasizes the rarity of alveolar hemorrhage as an initial symptom of Wegener's granulomatosis and the necessity of aggressive management.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
9/14. Microscopic polyarteritis: a forgotten aetiology of haemoptysis and rapidly progressive glomerulonephritis.A 76 year old white woman died from massive pulmonary haemorrhage nine days after she was admitted to Parkland Memorial Hospital for evaluation of haemoptysis and rapidly progressive glomerulonephritis. The differential diagnosis of haemoptysis with rapidly progressive glomerulonephritis is presented with particular emphasis on Wegener's granulomatosis and microscopic polyarteritis. Necropsy showed a small vessel necrotising vasculitis associated with a focal segmental necrotising glomerulonephritis consistent with microscopic polyarteritis.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
10/14. Wegener's granulomatosis presenting during first trimester of pregnancy.We describe a case of Wegener's granulomatosis in a lady who presented acutely with pulmonary haemorrhage, fever and breathlessness during her early pregnancy. She responded well to aggressive medical treatment.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
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