Gene/Protein Disease Symptom Drug Enzyme Compound
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 51,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Takayasu's arteritis (TA), also known as the "pulseless disease," is a chronic vasculitis of the aorta and aortic branches. TA with Crohn's disease is rare and has not been documented in China before. In this paper we report on a case of Takayasu's arteritis associated with concurrent Crohn's disease. A 17-year-old Chinese male developed upper limb sourness and a sensation of fatigue, and his upper limb pulses were absent. He was diagnosed with TA and underwent an axillary artery bypass with autologous great saphenous vein on the left subclavian artery. After the surgery, he regained the normal blood pressure. This patient also had years of diarrhea and developed an anal canal ulcer, and was diagnosed with inflammatory bowel disease and ulcerative colitis before. Five months after the TA surgery, he was hospitalized for severe stomachache and diarrhea and was finally diagnosed with Crohn's disease. The possible pathophysiological mechanisms responsible for concurrent existence of TA and Crohn's disease may be associated with immune disorders, especially autoimmunity.
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PMID:Takayasu's arteritis associated with Crohn's disease. 1965 Feb 3

We report a case of bacterial aneurysm complicated by severe infectious endocarditis. A 34-year-old man developed idiopathic fever and general fatigue persisting for a month. He was admitted to our institution, and examinations revealed severe bacterial endocarditis with vegetation at the mitral valve and mitral incompetence. Right after admission, he suddenly developed acute cardiac infarction and cardiac arrest due to occlusion of the coronary artery by emboli from vegetation of the mitral valve. After achieving a good recovery, magnetic resonance (MR) imaging demonstrated an unruptured bacterial aneurysm at the distal branch of the left middle cerebral artery (MCA) supplying the left parietal lobe 5 days after admission, and T2* weighted images demonstrated multiple signal loss lesions, which were suspected of being thrombosed bacterial micro-aneurysms or micro-vasculitis. Although there was a risk of aneurysm rupture, we decided to proceed with mitral valve replacement by an artificial heart valve made of carbon, and repeatedly observed an unruptured bacterial aneurysm by serial MR imaging and angiography. Due to the preceding cardiac surgery, we were able to completely cure the severe infection and prevent new embolic showers. Under administration of antibiotics, the bacterial cerebral aneurysm did not increase over a period of 4 weeks, and finally the aneurysm disappeared about 6 weeks after admission. Although the timing of treatment of an unruptured bacterial aneurysm and cardiac surgery for infectious endocarditis associated with a bacterial cerebral aneurysm are controversial, we think that proceeding with cardiac surgery and observing the unruptured bacterial aneurysm by repeated MR imaging and angiography under administration of antibiotics was an appropriate strategy in this case.
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PMID:[A case of bacterial aneurysm complicated by severe infectious endocarditis]. 1966 40

Large vessel vasculitis leads to arterial wall thickening and stiffening because of chronic inflammatory changes. The cardio-ankle vascular index (CAVI) is recently utilized for assessing arterial stiffening caused by atherosclerosis-related diseases, including hypertension and diabetes, as well as aging. CAVI is mathematically calculated from stiffness index beta, which is established as a parameter of arterial stiffness independent of blood pressure. However, there are no data regarding arterial stiffness assessed by CAVI for large vessel vasculitis. We describe a patient with large vessel vasculitis who showed aortic wall thickening and increased CAVI without hypertension. A 68-year-old woman presented at our hospital with recurrent fever of 2-month duration, fatigue, neck pain, and weight loss. The images of 18F-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) demonstrated significant 18FDG uptake (indicating increased metabolic activity and presence of inflammation) in the aorta and its major branches, including the carotid and subclavian arteries. Contrast-enhanced magnetic resonance imaging demonstrated wall thickening of the thoracic aorta. These imaging findings resulted in the diagnosis of large vessel vasculitis. The patient showed normal brachial blood pressure (right, 122/72 and left, 121/66 mmHg). However, CAVIs on both sides (right, 10.3 and left, 10.4) were elevated (normal value for her age, 9.1 +/- 0.8). In conclusion, arterial stiffness in patients with large vessel vasculitis may be increased because of the arterial wall thickening and inflammatory changes. Thus, CAVI may be promising for detection of increased arterial stiffness in patients with large vessel vasculitis in the early stage, in which blood pressure is normal.
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PMID:Detection of increased arterial stiffness in a patient with early stage of large vessel vasculitis by measuring cardio-ankle vascular index. 1977 26

Dapsone has potent anti-inflammatory effects, and is used in the treatment of leprosy, cutaneous vasculitis, neutrophilic dermatoses, and dermatitis herpetiformis and other blistering disorders. However, it may cause severe adverse reactions such as hypersensitivity syndrome, which is characterized by fever, skin rash, hepatitis and lymphadenopathy. We report a 44-year-old female Korean patient with dapsone hypersensitivity syndrome (DHS) that presented as a bullous skin eruption. The patient had a 1-year history of urticarial vasculitis, treated with antihistamines, prednisolone and dapsone. Although the skin lesions improved, she reported fever, nausea, abdominal pain, jaundice, fatigue and skin rashes. On physical examination, there were generalized erythematous macules and purpura with facial oedema that developed into vesicles on the upper limbs. Histological examination of a skin biopsy of a vesicular lesion found subepidermal oedema with a mixed inflammatory cell infiltrate, including eosinophils in the dermis. Indirect immunofluorescence testing using normal foreskin as substrate revealed IgG deposits in the basement membrane zone. Circulating autoantibodies against antigens of 190 and 230 kDa were found by immunoblotting analysis using epidermal extracts. This case illustrates DHS with the formation of circulating autoantibodies.
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PMID:Dapsone hypersensitivity syndrome with circulating 190-kDa and 230-kDa autoantibodies. 1977 10

Wegener's granulomatosis is a type of vasculitis characterized by necrotizing granulomatosis respiratory tract lesions and necrotising glomerulonephritis. Nasal, lung and renal biopsies and positive antineutrophil cytoplasmic antibody (c-ANCA) analysis is helpful in diagnosis. Early diagnosis and treatment is critical in prognosis. A 42 years-old male had dyspnea, coughing, hemoptysis, fatigue, loss of appetite, night sweating and arthralgia. Violaceous palpable, purpuric lesions were detected on the sublingual region of the mouth. On chest X-ray, there was nonhomogeneous infiltration in the parenchyma of both lungs. There was alveolar density involving upper lobes of both lungs detected in thoracal computerized tomography as well as patchy densities on the right lung upper-middle lobes. A nasal mucosa biopsy showed strongly destructive vasculitis. c-ANCA test was positive. We report an unusual case with Wegener's granulomatosis, characterized by a rare presentation of tongue involvement and atypical lung radiology with alveolar opacity.
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PMID:An unusual case of Wegener's granulomatosis with tongue involvement. 1978 77

In early June 2004, a 50-year-old female was admitted to the hospital for slight fever, general fatigue, hemoptysis, dyspnea, and renal dysfunction (serum creatinine[Cr] : 6.05 mg/dL). She had been treated with prednisolone (PSL : 10-20 mg/day) for RA. She was diagnosed with Goodpasture syndrome based on a high titer of anti-glomerular basement membrane antibody (87 EU), and pulmonary hemorrhage. The renal and pulmonary impairments were markedly improved by the pulse therapy, plasma exchange and temporary hemodialysis. However, the Cr level remained at 2.0 mg/dL, indicating nephrotic syndrome. Light microscopy with Periodic acid-Shiff(PAS) staining demonstrated global sclerosis in three of ten glomeruli. Five glomeruli showed the formation of cellular, and fibrocellular crescents, and the formation of fibrous crescents. Tubular damage and interstitial fibrosis were severe. Immunofluorescence microscopy disclosed major depositions of IgG in a linear pattern along the glomerular basement membrane(GBM). Electron microscopy revealed foot process effacement (>50%)and no electron-dense deposits. Therefore, we diagnosed Goodpasture syndrome associated with minimal change nephrotic syndrome (MCNS). Some reports have dealt with the association of RA and Goodpasture syndrome with D-penicillamine, and of RA and antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis with pulmonary hemorrhage, but none has dealt with cases complicated with RA and Goodpasture syndrome associated with MCNS. Accordingly, whether or not there is a causal relationship between RA and Goodpasture syndrome remains obscure, but since the number of reported cases is small, experience with more cases is necessary to clarify this matter.
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PMID:[Case of goodpasture syndrome associated with minimal change nephrotic syndrome (MCNS) in a patient with rheumatoid arthritis (RA)]. 1992 65

Propylthiouracil (PTU) is a frequently prescribed drug in the treatment of hyperthyroidism. The use of PTU is, however, accompanied by numerous potentially serious side effects including vasculitis. PTU-related vasculitides can present as haematuria, pulmonary haemorrhage, or cutaneous lesion together with aspecific symptoms such as fever, myalgia, arthralgia, and fatigue. Cerebral involvement is seldom observed. We present a 49-year-old female with Graves' disease and asthma, who developed paresis of the proximal extremities, eosinophilia, pulmonary, and cutaneous lesions following treatment with PTU. A cerebral vasculitis consistent with Churg-Strauss syndrome (CSS) was suspected. Although cerebral involvement is seldom observed with PTU treatment, cerebral vasculitis should be considered in patients developing CNS symptoms.
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PMID:Churg-Strauss Syndrome following PTU Treatment. 2010 67

Systemic vasculitides are a heterogenous group of chronic relapsing diseases. Initial data show that health-related quality of life (HRQoL) from the patient's perspective is reduced on all levels (physical, social and emotional). These studies suggest that generic questionnaires do not encompass all relevant and important levels of HRQoL in vasculitis. Moreover, data indicate that tiredness (fatigue) and reduced energy levels play a significant role. This article presents an overview of the existing literature.
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PMID:[The significance of health-related quality of life in systemic vasculitides]. 2030 2

Aortitis syndrome is a chronic vasculitis that leads to arterial wall thickening and stiffening in large elastic arteries. However, there are no established markers for assessing arterial stiffening in aortitis syndrome. The cardio-ankle vascular index (CAVI) has recently been utilized to assess arterial stiffening that is associated with atherosclerosis-related diseases. We hypothesized that CAVI can be applicable for assessing alterations in arterial stiffness during immunosuppressive therapy for aortitis syndrome. A 69-year-old woman with a 2-month history of recurrent fever, fatigue, and malaise, showed intense 18F-fluorodeoxyglucose (18F-FDG) uptake in the thoracic aorta and common carotid arteries in 18F-FDG-positron emission tomography. These clinical and imaging findings resulted in the diagnosis of aortitis syndrome. The patient also showed the elevated CAVIs on both sides (right, 10.3; left, 10.4) (normal value for her age, 9.1 +/- 0.8), indicating the arterial stiffness due to aortitis syndrome. The patient was treated for 34 weeks with immunosuppressive therapy, which included oral prednisolone and methotrexate. C-reactive protein (from 4.24 to 0.49 mg/dL) and immunoglobulin G (from 2,627 to 1,524 mg/dL) were decreased by 7 weeks after initiation of the treatment. The decrease in these inflammatory parameters suggests the effectiveness of the immunosuppressive therapy. In addition, after the 34-week treatment, the CAVIs on both sides (right, 9.3; left, 9.2) were within the normal range. These data indicate that the immunosuppressive therapy ameliorates the degree of arterial stiffness. In conclusion, CAVI may be a promising marker for evaluating the effectiveness of immunosuppressive therapy in patients with aortitis syndrome.
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PMID:Cardio-ankle vascular index for evaluating immunosuppressive therapy in a patient with aortitis syndrome. 2082 65

The patient was an 87-year-old man whose initial symptom was general fatigue and inappetence. His laboratory data revealed a rise in C-reactive protein (CRP) and white blood cell count (WBC), and CT scan showed suspicious pneumonia. Antibiotics were given to the patient, but his fever and laboratory data were sustained. Follow up examination revealed a high titer (107 U/ml) of myeloperoxidase specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA). He was diagnosed with MPO-ANCA associated vasculitis. Steroid pulse therapy was started. The patient's clinical symptoms and laboratory data thereafter significantly improved, but after one week the patient's symptom was aggravated and he died. An autopsy was performed, and necrotizing arteritis of the interlobular arteries were found in the kidneys. We found bacterial infective vegetation attached to the aortic valve. Infected thromboembolus and microabscesses were also found in many organs. We report a case of subacute microscopic polyangiitis associated with bacterial endocarditis.
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PMID:[An autopsy case of microscopic polyangiitis associated with bacterial endocarditis]. 2085 21


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