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Query: UMLS:C0010200 (cough)
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Wegener's granulomatosis is a form of systemic vasculitis of unknown etiology that affects the small blood vessels. The disease is characterized by the formation of granulomas on small and medium blood vessels, and often affects the respiratory tract and renal system. In 86% of diagnosed cases, renal compromise is in the form of glomerulonephritis. Wegener's granulomatosis can occur at any age, principally during the 4th and 5th decades of life, and 85% of cases occur over the age of 19 years. In the United States about 3 per 100,000 cases are reported, with a major occurrence in males. The present report is about a case of a 12 year old female with Wegener's granulomatosis of 18 months of evolution, with symptoms such as epistaxis, generalized edema, cough, dyspnea, hemoptysis, high blood pressure and macroscopic hematuria. Out of the five criteria for the diagnosis of Wegener's granulomatosis, she had perinuclear anti-neutrophil cytoplasmic antibody positive to serum p-ANCA mark, which is the least frequent, which makes more difficult the diagnosis. Over a period of five years, in our hospital, we have diagnosed 11 patients with Wegener's granulomatosis, and the present case is the only pediatric case.
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PMID:[Wegener's granulomatosis. Report of a pediatric case and review of the literature]. 1282 92

A non-smoking 76-year-old woman was admitted to our hospital complaining of cough, bloodstained sputum and difficulty in breathing that had developed while she was eating a meal. The initial diagnosis was upper airway obstruction. Her past history revealed no major illness, except that she was under medication for the treatment of hypertension. On examination, chest radiography and computed tomography (CT) scans showed diffuse ground glass opacities in both lungs, and bronchoalveolar lavage studies further yielded findings compatible with pulmonary hemorrhage. The patient's symptoms and hypoxemia improved gradually without any specific treatment. Laboratory data showed no specific underlying cause, such as vasculitis or collagen vascular disease, for the pulmonary hemorrhage. We therefore reached a diagnosis of a negative pressure pulmonary hemorrhage (NPPH) caused by upper airway obstruction. Follow-up examinations after 7 months showed no abnormal findings in the chest radiography or arterial blood gas analysis. To our knowledge, this is the first case of negative pressure pulmonary hemorrhage in japan.
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PMID:[A case of negative pressure pulmonary hemorrhage (NPPH) due to upper airway obstruction induced by asphyxia]. 1283 47

We present a case of allergic rhinitis in a 68-year-old woman in whom eosinophilia was found when she complained of common cold-like symptoms. The patient noticed a mass lesion on her left neck, which improved with antibiotic treatment, but her coughing continued and edema of both lower extremities appeared. She was admitted to our hospital, because of abnormalities in her electrocardiogram and cardiomegaly seen in a chest radiograph. The discomfort due to the edema in the soles of both feet remained even after steroid therapy. Her chest radiograph revealed ground-glass opacity, and a transbronchial lung biopsy revealed granulation tissue with the infiltration of eosinophils into the interstitium. Allergic granulomatosis angiitis was diagnosed because of granulomatosis vasculitis resulting from sural nerve biopsy. This was a rare case of allergic granulomatosis angiitis because her lung function was normal, she had no history of bronchial asthma, and there were no clear symptoms of bronchial asthma.
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PMID:[A case of allergic granulomatosis and angiitis without symptoms of asthma]. 1453 2

Non-specific interstitial pneumonia developed as an initial manifestation in a patient with microscopic polyangiitis. A 62-year-old man was admitted to our hospital in March 2001, because of fever and intermittent myalgia of lower extremities. Chest X-ray had revealed reticular shadows in the bilateral middle and lower lung fields since 1996. Just before admission, the patient had been diagnosed as having nonspecific interstitial pneumonia (NSIP) from the specimen obtained by video-assisted thoracoscopic surgery (VATS) in another hospital. Physical examination on admission revealed bilateral episcleritis. Laboratory data showed elevated levels of CRP and KL-6, polyclonal gammaglobulinemia, positive rheumatoid factor and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). Sensory and motor nerve conducting velocities were delayed in left peroneal nerve, but not other nerves, suggesting mononeuropathy. Biopsied specimens of the left quadriceps revealed vasculitis of arteioles. In spite of positive proteinuria and hematuria, no pathological lesion was found in the kidney. From all of these findings, the patient was diagnosed as having microscopic polyangiitis (MPA) without renal involvement. Methylprednisolone pulse therapy followed by intravenous cyclophosphamide pulse therapy improved his clinical conditions such as pyrexia, cough, myalgia, episcleritis and respiratory symptoms with decreased titer of serum MPO-ANCA. Thereafter, the dose of prednisolone was successfully tapered to 10 mg/day without clinical relapse. In the present patient who developed demonstrated non-specific interstitial pneumonia as an initial manifestation of MPA, VATS provided useful diagnostic and prognostic information, leading to an appropriate therapeutic choice.
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PMID:[A case of non-specific interstitial pneumonia in patient with microscopic polyangiitis]. 1459 58

A 50-year-old Japanese man with Grave's disease had been taking propylthiouracil (PTU) for 10 years prior to the diagnosis of pneumonia. He noticed dyspnea on exertion and had a dry cough for at least 2 years and then suddenly developed high fever and dyspnea at rest. Clinical symptoms, chest radiographs, chest computed tomography and lung function revealed interstitial pneumonia. The symptoms were completely resolved after withdrawal of PTU, and consequently he was diagnosed with PTU-induced interstitial pneumonia. He also showed moderate myeloperoxidase (MPO)- antineutrophil cytoplasmic antibody (ANCA) positivity without any signs of vasculitis before as well as after PTU withdrawal. Although PTU-induced interstitial pneumonia is quite rare, with only 3 cases, including the present patient, so far reported, respiratory involvement should be considered in patients treated with PTU.
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PMID:A man with interstitial pneumonia due to propylthiouracil. 1460 20

Allergic granulomatosis and angiitis, also known as Churg-Strauss syndrome (CSS), is an uncommon vasculitis of unknown etiology. We report a 21-year-old male patient with fatigue, dry cough, and progressive dyspnea. He had no history of asthma or eosinophilia. Thorax computed tomography showed bullous/cystic areas with thin walls in varying sizes (5-15 mm). Histopathological examination of the open lung biopsy revealed granulomatous infiltration with histiocytes and eosinophilic leukocytes. This extremely rare variant of CSS is discussed.
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PMID:Allergic granulomatosis and angiitis in the absence of asthma and blood eosinophilia: a rare presentation of limited Churg-Strauss syndrome. 1462 52

A 33 years old woman was admitted to the hospital after four days with cough, dyspnea, orthopnea and hemoptysis. Blood pressure was 170/90 mmHg, pulse was 112 and temperature was normal. She had cyanosis and a left ventricular gallop, without heart murmurs. A chest radiograph revealed pulmonary edema and echocardiogram showed a global left ventricular systolic disfunction. Oxygen and furosemide were started, but cardiopulmonary collapse ensued. The patient was supported with mechanical ventilation and treated with inotropic drugs. A right sided cardiac catheterization showed pulmonary wedge pressure of 18 mmHg and a cardiac index of 3 l/min/m2. The levels of creatinine and urea nitrogen were elevated and a urine protein was 97 mg/dl. Coagulation tests were normal except by a positive lupic anticoagulant. Markers of connective tissue diseases or vasculitis were negatives. The clinical evolution suggested that a catastrophic antiphospholipid syndrome was ongoing. Intravenous corticoids, gammaglobulin and cyclophosphamide were administered with transient improvement. On her fourth day of treatment, the patient presented sudden pulmonary bleeding and embolism. A plasmapheresis was performed with improvement of renal, cardiac and pulmonary function. After this episode, the patient has been treated with prednisone and oral anticoagulants treatment for the last two years, without further clinical events.
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PMID:[Catastrophic antiphospholipid syndrome and acute heart failure. Report of a case]. 1463 91

A 46-year-old woman was admitted to our hospital because of fever, cough and headache in December 2001. Although she had been treated for nasal obstruction and epistaxis by an otorhinolaryngologist in our hospital since 1996, no accurate diagnosis had been made despite repeated biopsies of the nasal mucosa. A chest CT taken in 1999 showed ground-glass opacities in both upper lobes. On admission, chest radiography and CT showed mass shadows without cavitation, corresponding to the lesions causing the ground-glass opacities. In addition, paranasal sinus MRI showed a deformity of the nasal septum accompanied by a space-occupying lesion, suggesting Wegener's granulomatosis. However, the cytoplasmic-antineutrophil cytoplasmic antibody (c-ANCA) test was negative. To achieve a definitive diagnosis, we performed an open lung biopsy. The specimen, obtained from the right upper lobe, showed the typical findings of a Wegener's granulomatosis including necrotizing vasculitis. Oral prednisolone treatment initiated at 20 mg daily, combined with oral cyclophosphamide at 50 mg daily markedly improved not only the clinical symptoms, but also the mass shadows in the left upper lobe. Patients with the limited form of Wegener's granulomatosis are occasionally seronegative and respond well to therapy. However, the natural course and the changes in chest radiographs are not understood well in such cases. In this paper, we report a case of the limited form of Wegener's granulomatosis that progressed slowly over a period of 6 years.
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PMID:[A case of the limited from of Wegener's granulomatosis without c-ANCA]. 1466 58

We present a 7-year-old boy who was admitted with a history of cough for a week, neck pain with associated swelling for 4 days, fever, and vague periumbilical pain. He was diagnosed with Kawasaki disease, and subsequently developed vasculitis of the ureter and stricture of the ureteric lumen at the level of the pelviureteric junction.
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PMID:Pelviureteric junction obstruction as sequelae of Kawasaki disease. 1524 77

A 53-year-old man was referred to our hospital because of atypical cells in the peripheral blood. Myelodysplastic syndrome (MDS) was diagnosed by bone marrow examination. Since he had a continuous dry cough, high fever, and air space consolidation in both lower lung fields, he was admitted to our hospital for further examination. Infectious disease, autoimmune disease, vasculitis and neoplasms were excluded. A transbronchial lung biopsy (TBLB) was performed at the right S(10), and histopathological examination of the specimen thus obtained suggested an organizing pneumonia (OP) associated with MDS. Oral prednisolone 30 mg was administered daily, and the symptoms and radiographic findings immediately improved. Accurate diagnosis of the pulmonary involvement associated with patients with MDS is sometimes difficult to make. However, either BOOP or OP should be considered as a differential diagnosis in case the patient presents with continuous coughing, high fever, and air space consolidation. Interventional examinations such as TBLB should be conducted to make a histological diagnosis if the patient's condition allows it.
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PMID:[A case of organizing pneumonia associated with myelodysplastic syndrome (MDS)]. 1535 71


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