Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0206061 (interstitial pneumonia)
 6,105 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

[Case 1]: An 81-year old man was referred to our hospital with dyspnea and bloody sputum. Computed tomography with contrast medium for the evaluation of metastasis of urinary bladder carcinoma had been performed 4 months previously. On admission, his serum creatinine and potassium were 15.3 mg/dl and 6.9 mEq/l, respectively. His chest X ray revealed cardiomegaly, butterfly shadow and interstitial change, indicating congestive heart failure and interstitial pneumonia. His electrocardiogram showed that he was on the brink of cardiac arrest due to hyperkalemia. Mechanical ventilation and hemodialysis were initiated. Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) was highly positive(321 EU), leading to the diagnosis of MPO-ANCA-associated rapidly progressive glomerulonephritis (RPGN) with interstitial pneumonia. Treatment with pulse methylprednisolone was not effective and he died. Autopsy findings showed crescentic glomerulonephritis, alveolar hemorrhage and interstitial pneumonia with honeycomb formation. [Case 2]: A 73-year old man was referred to our hospital with rapid deterioration of his renal function. He had received a cardiac catheter examination 3 weeks previously. On admission, his serum creatinine was 4.5 mg/dl. His chest X ray showed cardiomegaly and interstitial change. Renal biopsy findings showed crescentic formation in the glomeruli. Moreover, MPO-ANCA was 494 EU, leading to the diagnosis of MPO-ANCA-associated RPGN with interstitial pneumonia. Treatment with pulse methylprednisolone and cyclophosphamide was not effective and he died. Autopsy findings revealed crescentic glomerulonephritis and interstitial pneumonia with honeycomb formation. Here we described two cases of ANCA-associated RPGN complicated by microscopic polyantitis and interstitial pneumonia after the use of contrast medium. The relation between ANCA-associated RPGN and the contrast medium was unclear. However, in the case of rapid deterioration of renal function, MPO-ANCA should be measured even after the use of contrast medium. The complication of lung diseases, especially interstitial pneumonia, should be investigated simultaneously.
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PMID:[Two autopsy cases of ANCA-associated glomerulonephritis manifested after contrast medium use]. 1677

Myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) is an autoantibody that is frequently found in patients with vasculitides. We encountered some MPO-ANCA positive patients with interstitial pneumonia who lacked vasculitides, but its meaning remains unclear. We measured MPO-ANCA titers in 69 patients with interstitial pneumonia (IP) who did not have collagen vascular diseases and observed their outcome. MPO-ANCA was positive in 5 patients and its prevalence was 7.2%. Patients with MPO-ANCA positive showed higher positivity in rheumatoid factor (RF) than patients with MPO-ANCA negative. The sensitivity and specificity of a combination of anti-nuclear antibody-negative and RF-positive were 80.0% and 87.7%, respectively. Two patients were accompanied by microscopic polyangiitis and the 3-year survival rate was 40% in all patients with MPO-ANCA. Measurement of MPO-ANCA titers in patients with IPs is meaningful for determining therapeutic strategy.
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PMID:[Prevalence of myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) in patients with interstitial pneumonia]. 1818 36

A 65-year-old man was admitted to our hospital because of progressive dyspnea. A laboratory examination and high-resolution computed tomography (HRCT) revealed that he had interstitial pneumonia (IP) with liver dysfunction. Myeloperoxidase-ANCA (MPO-ANCA) was negative. Although his respiratory condition had become stable after initiation of steroid therapy, liver dysfunction had worsened with progressive portal hypertension. He died of hepatic insufficiency about three years after the first medical examination. Autopsy showed that he had vasculitis of medium and small blood vessels of the spleen, lungs, and liver. The final diagnosis was classical polyarteritis nodosa (PAN). Microscopically, non-specific interstitial pneumonia was identified in the autopsied lung. The pathological findings of the liver were consistent with nodular regenerative hyperplasia (NRH). We report a case of PAN with IP and NRH preceding findings of systemic vasculitis.
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PMID:Interstitial pneumonia and nodular regenerative hyperplasia of the liver as initial manifestations of polyarteritis nodosa. 2244 74

Myeloperoxidase anti-neutrophil cytoplasmic autoantibody (MPO-ANCA) is a well known marker for small vessel vasculitis. Recent reports have demonstrated that interstitial pneumonia (IP) may rarely be associated with serum MPO-ANCA. Yet, little is known about the histological features. We reviewed surgical lung biopsy from nine patients with IP of uncertain etiology with serum MPO-ANCA. There was a male predominance (6:3) with a median age of 62.1. Histologically, eight patients presented with a usual interstitial pneumonia (UIP) pattern of pulmonary fibrosis, frequently accompanied by areas of nonspecific interstitial pneumonia (NSIP) pattern. One patient showed diffuse alveolar damage (DAD), and two patients showed mixture of UIP and DAD reflecting acute exacerbation of UIP. Microscopic honeycomb cysts were common, but fibroblastic foci were inconspicuous. The most frequent additional findings were small airway disease (9/9), and lymphoid follicles (7/9). Neither capillaritis nor vasculitis was seen in any of our cases. Three patients had microscopic hematuria, but none progressed to microscopic polyangiitis during the follow up. Mortality rate was 44% (median follow up 39.1 months). IP associated with MPO-ANCA showed characteristic histology dominated by UIP pattern. Vasculitis was not identified in our cohort, but small airways disease and lymphoid follicles were present in most cases. IP associated with MPO-ANCA may be a histologically distinctive disease from idiopathic pulmonary fibrosis. Mortality was relatively high and life threatening acute exacerbation may occur.
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PMID:Interstitial pneumonia associated with MPO-ANCA: clinicopathological features of nine patients. 2299 39

Myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA) is well-known as a serological marker for small-vessel vasculitis. However, when a smoker with interstitial lung disease (ILD) exhibits serum ANCA positivity without systemic vasculitis, diagnosis is a matter of debate; the relationship between smoking and ANCA is unknown. We report a case of combined pulmonary fibrosis and emphysema (CPFE) with elevated MPO-ANCA. Surgical lung biopsy showed emphysema and fibrotic interstitial pneumonia without vasculitis. The MPO-ANCA level decreased after smoking cessation, and no vasculitis or progression was observed during 3 years of follow-up. This suggested that smoking cessation was related to normalization of MPO-ANCA and corresponding disease activity.
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PMID:Combined pulmonary fibrosis and emphysema with myeloperoxidase-antineutrophil cytoplasmic antibody positivity that resolved upon smoking cessation. 3018 56