Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 21-year-old woman had suffered from repeated vasculitis and panniculitis with fever and chills after following mosquito bites since age 7. These manifestations were confirmed with a direct mosquito bite test during her admission to another hospital at age 17. The patient presented to our hospital with progressive dyspnea, productive cough and intermittent fever for one week. In addition to the bilateral infiltrative lesions on chest roentgenography, hepatosplenomegaly with an abnormal liver function test, pancytopenia, and elevated IgE were also detected. Blood and sputum cultures grew no microorganisms. Epstein-Barr virus-IgM, Cytomegalovirus-IgM and Mycoplasma pneumonia antibodies were all negative. Bone marrow aspiration and biopsy revealed histiocytosis with hemophagocytosis. No atypical histiocyte was found. The patient was put on dexamethasone treatment with improvement. Unfortunately, symptoms relapsed two weeks later. A repeated bone marrow aspiration and biopsy revealed a picture similar to the previous one. Despite antibiotic administration and ventilator support, a rapidly deteriorated course terminated in the patient's death by respiratory failure. Mosquito bite allergies were reported to be associated with malignant histiocytosis in Japan. However, atypical histiocytes were not found in our case upon repeated bone marrow aspirations, biopsies and skin biopsies. Mature histiocytes with hemophagocytosis were prominent instead. Reactive histiocytosis was thus favored rather than malignant histiocytosis. Although opportunistic infection cannot be excluded, we propose that a mosquito bite allergy with consequent histiocytic activation by antigen, immune complex or IgE is the possible pathogenetic mechanism for hemophagocytic histiocytosis in this patient.
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PMID:[Mosquito bite allergies terminating as hemophagocytic histiocytosis: report of a case]. 257 73

A 66-year-old woman, who had had bronchial asthma, was admitted to our hospital because she suffered from fever, productive cough, wheezing, dyspnea, and chest pressure sensation. Her chest X-ray showed migrating infiltration and marked cardiomegaly. Her ECG at the admission revealed abnormal Q wave and T wave inversion, though that of 3 years before had been almost normal. Hematology showed leukocytosis and eosinophilia of 8,000/mm3 without abnormal cells. All immunological tests were negative and the specific cause of the eosinophilia was unknown. 2 weeks after admission, she complained of severe chest pain suddenly and her ECG showed ST elevation on V1-4 and serum CPK level was elevated to 290 IU/l. By the thrombolytic agent and anticoagulant therapy, her symptom was lightened immediately. 2 months later, we made her cardiac catheterization and myocardial biopsy. Her LVG showed a small aneurysm of the apex, though her CAG was normal finding. The biopsy revealed moderate fibrosis and cellular infiltration including a few eosinophils. We thought that eosinophilic endocarditis had existed first, and secondary embolism continued led to the small infarction. The hypereosinophilia was spontaneously normalized 2 months after admission, but the patient complained of myalgia and sensory disturbance of extremities. The biopsy of quadriceps muscle could prove neither infiltration of eosinophils nor vasculitis. But we diagnosed mononeuritis multiplex due to hypereosinophilia. Judging from various symptoms and laboratory findings, this case was included to the hypereosinophilic syndrome. We also thought allergic granulomatosis and angitis as one of the differential diagnoses, but histologically vasculitis was not proved. In this case, eosinophilia was disappeared without using corticosteroids.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of hypereosinophilic syndrome associated with eosinophilic endocarditis]. 261 22

A 32-year-old female complained of productive cough and bloody sputum. Infiltrative shadows and cavitary lesions with thick and irregular wall in the bilateral lung fields and the swelling of mediastinal lymph nodes were pointed out on the chest radiography. Physical examination revealed no abnormal findings. ESR and the level of the serum angiotensin converting enzyme were slightly elevated. In the BAL (broncho-alveolar lavage) fluid, lymphocytes and neutrophils increased, and the OKT4/OKT8 ratio of the lymphocytes was 0.81. Open lung biopsy revealed numerous sarcoid granulomas with granulomatous vasculitis in the cavity wall, surrounding infiltrative lesions and hilar lymph nodes. After the administration of prednisolone, the infiltrative shadows and the cavitary lesions showed marked improvement. It was concluded that open lung biopsy is necessary for the diagnosis of NSG because the differential diagnosis between NSG and limited form of Wegener's granulomatosis is extremely difficult from such a small lung specimen as that obtained by trans-bronchial lung biopsy.
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PMID:[A case of necrotizing sarcoid granulomatosis diagnosed by open lung biopsy]. 274 62

We report two cases of Churg-Strauss syndrome with impaired prognosis. Case 1: A 58-year-old woman started to complain of progressive dysesthesia and muscle weakness of the lower extremities on February 6, 1992 and was admitted to our hospital eight days later. The eosinophil count was 1, 170/microliters and chest X-ray on admission revealed bilateral diffuse interstitial shadows. Necrosis of muscle fibers and significant infiltration of eosinophils into vessel walls were histopathologically demonstrated. Case 2: A 69-year-old woman was admitted to our hospital with productive cough and back pain. An expiratory wheeze was audible in all lung fields and bronchial asthma was diagnosed. Laboratory findings showed eosinophilia (8, 550/microliters), an IgE level of 16, 266 IU/ml and no positive data for allergic bronchopulmonary aspergillosis. A biopsy of subcutaneous nodules in the lower extremity revealed significant edema of vessel walls and infiltration eosinophils and lymphocytes. Churg-Strauss syndrome was diagnosed in both cases, and corticosteroid therapy was successful in alleviating eosinophilia. However, symptoms of vasculitis in case 1, including mononeuritis multiplex, did not improve, and cardiomyopathy in case 2 progressively worsened. Particularly in elderly patients, it is very important to make an early diagnosis and to initiate therapy that does not disturb quality of life, even for diseases generally regarded as having a good or fair prognosis.
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PMID:[Two cases of Churg-Strauss syndrome with impaired prognosis]. 974 2

Given the variability in rate of radiographic resolution, it remains controversial to decide when to initiate an invasive diagnostic work-up for nonresolving or slowly resolving pulmonary infiltrates. In immunocompetent patients who present with classical features of CAP (i.e., fever, chills, productive cough, new pulmonary infiltrate), clinical response to therapy is the most important determinant for further diagnostic studies. Within the first few days, persistence or even progression of infiltrates on chest radiographs is not unusual. Defervescence, diminished symptoms, and resolution of leukocytosis strongly support a response to antibiotic therapy, even when chest radiographic abnormalities persist. In this context, observation alone is reasonable, and invasive procedures can be deferred. Serial radiographs and clinical examinations dictate subsequent evaluation. In contrast, when clinical improvement has not occurred and chest radiographs are unchanged or worse, a more aggressive approach is warranted. In this setting, we advise fiberoptic bronchoscopy with BAL and appropriate cultures for bacteria, legionella, fungi, and mycobacteria. When endobronchial anatomy is normal and there is no purulence to suggest infection, TBBs should be done to exclude noninfectious causes (discussed earlier) or infections attributable to mycobacteria or fungi. An aggressive approach is also warranted in patients who are clinically stable or improving when the rate of radiographic resolution is delayed. As discussed earlier, what constitutes excessive delay is controversial, and depends upon the acuity of illness, specific pathogen, extent of involvement (i.e., lobar versus multilobar), comorbidities, and diverse host factors. Stable infiltrates even 2 to 4 weeks after institution of antibiotic therapy does not mandate intervention provided patients are improving clinically. Invasive techniques can also be deferred when unequivocal, albeit incomplete, radiographic resolution can be demonstrated. Lack of at least partial radiographic resolution by 6 weeks, even in asymptomatic patients, however, deserves consideration of alternative causes (e.g., endobronchial obstructing lesions, or noninfectious causes). Fiberoptic bronchoscopy with BAL and TBBs has minimal morbidity and is the preferred initial invasive procedure for detecting endobronchial lesions or substantiating noninfectious causes. The yield of bronchoscopy depends on demographics, radiographic features, and pre-test likelihood. In the absence of specific risk factors, the incidence of obstructing lesions (e.g., bronchogenic carcinomas, bronchial adenomas, obstructive foreign body) is low. Bronchogenic carcinoma is rare in nonsmoking, young (< 50 years) patients but is a legitimate consideration in older patients with a history of tobacco abuse. Non-neoplastic causes (e.g., pulmonary vasculitis, hypersensitivity pneumonia, etc.) should be considered when specific features are present (e.g., hematuria, appropriate epidemiologic exposures). Ancillary serologic tests or biopsies of extrapulmonary sites are invaluable in some cases. In rare instances, surgical (open or VATS) biopsy is necessary to diagnose refractory or non-resolving "pneumonias."
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PMID:Nonresolving or slowly resolving pneumonia. 1051 9

A 53-year-old woman was referred to our hospital with the main symptoms of productive cough, fever and exertional dyspnoea. Chest X-ray revealed enlargement of the left hilar shadow and cavitary infiltration in the right upper lobe. 99mTechnetium-macroaggregated albumin (99mTc-MAA) perfusion scintigram showed complete hypoperfusion through the entire right lung. A pulmonary angiogram revealed stenotic lesions in the right and left main pulmonary arteries. Right cardiac catheterization showed an elevated right ventricular systolic pressure. There was no evidence of systemic arterial lesions nor vasculitis. The patient was positive for myeloperoxidase (MPO)-antineutrophil cytoplasmic autoantibodies (ANCA) (168 EU). The Mycobacterium avium complex sputum culture was positive. The pulmonary stenotic lesions were surgically resected. The resected pulmonary arterial lesions were pathologically diagnosed as non-specific vasculitis. The cavitary lesion disappeared 6 months after the surgery. Two years after the surgery, although the MPO-ANCA level had decreased to 12 EU, stenosis of the pulmonary arteries reappeared. It is suggested that the patient became positive for MPO-ANCA in association with the Mycobacterium avium complex infection, and that the presence of MPO-ANCA may not be related to the development of pulmonary stenosis of the main pulmonary arteries.
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PMID:A case of pulmonary arteritis with stenosis of the main pulmonary arteries with positive myeloperoxidase-antineutrophil cytoplasmic autoantibodies. 1119 50

A 72-year-old woman was admitted complaining of productive cough. She had worked for an asbestos factory for twenty years. She was positive for MPO-ANCA. The chest HRCT showed interstitial pneumonia without any UIP pattern. A specimen obtained by video-assisted thoracoscopic lung biopsy revealed chronic interstitial pneumonia associated with an asbestos body. Although the interstitial pneumonia improved after the administration of a corticosteroid and an immunosuppressive agent, hematuria and renal dysfunction developed about nine months later. The serum MPO-ANCA titer was elevated and the renal biopsy specimen revealed the presence of vasculitis. As the interstitial pneumonia improved after this treatment, the correct diagnosis may have been, not asbestosis, but MPO-ANCA-related interstitial pneumonia.
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PMID:[A case of MPO-ANCA-related vasculitis after asbestos exposure with progression of a renal lesion after improvement of interstitial pneumonia]. 1522 36

A 66-year-old woman was referred a 4-month history of asthenia, weight loss, productive cough, increasing dyspnea, epigastric pain, and night sweats. A B-cell non-Hodgkin lymphoma was discovered in association with a severe leukocytoclastic vasculitis and disseminated intravascular coagulation. The patient was treated successfully with the combination of cyclophosphamide, vincristine, prednisone, and rituximab.
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PMID:Generalized B-cell non Hodgkin's lymphoma in association with leukocytoclastic vasculitis and disseminated intravascular coagulation. 1663 29

A 57-year-old man who had a history of sinusitis was admitted to Ryugasaki-Saiseikai hospital in April 2002 because of productive cough and bloody sputum. Chest radiographs and CT scans showed mediastinal lymphadenopathy and a solitary mass lesion with an irregular margin and cavity in the left lower lung field. Proteinase 3 antineutrophil cytoplasmic antibody (PR3 ANCA) was positive, and this is a sensitive and specific indicator of Wegener's granulomatosis. The pathological findings from transbronchial biopsy revealed squamous cell carcinoma of the lung, without the presence of vasculitis, accompanied by Wegener's granulomatosis. A partial response was finally obtained after three courses of paclitaxel and carboplatin. The serum level of PR3 ANCA decreased from 142 EU to 16 EU. This case appears to have had parallel time courses of progression of squamous cell carcinoma of the lung and changes in serum PR3 ANCA level. This is of importance in considering the relationship of lung cancer and paraneoplastic vasculitis.
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PMID:[A case of squamous cell carcinoma of the lung with a high titer of proteinase 3 antineutrophil cytoplasmic antibody]. 1722 9

We report the first case of pulmonary Corynebacterium ulcerans infection mimicking Churg-Strauss syndrome (CSS). Productive cough, fever, general fatigue, and weight loss developed in a 50-year-old man. Laboratory data revealed prominent eosinophilia and elevated serum IgE. On chest images, multiple nodules and cavities were predominantly detected in the right lung. Histopathologic examination showed necrotizing granulomas and vasculitis with massive eosinophilic infiltration identical to the findings seen in CSS; however, clusters of Gram-positive, coryneform rods were observed in the alveolar spaces. A toxigenic strain of C ulcerans was isolated from lung tissue. The patient was treated with antibiotics, and a favorable clinical course ensued.
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PMID:Corynebacterium ulcerans infection of the lung mimicking the histology of Churg-Strauss syndrome. 1742 34


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