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)

The pathological findings in the lungs and related organs of 26 patients (21 female, 5 male) with systemic lupus erythematosus (SLE), with onset of disease before age 20 years, were reviewed. Several categories of lung lesions were found. Chronic interstitial pneumonitis was present in all 26 patients and was severe in 5. Acute pneumonia was present in 20, mild in 13, moderate in 2, and severe in 5. Alveolar hemorrhage, massive enough to cause death in 5, was seen in 18 patients, and pulmonary edema was found in 13. Fourteen patients had hyaline membranes indicative of acute alveolar damage (DAD), 12 had alveolitis obliterans, indicative of prior episodes of DAD, and 9 had bronchiolitis obliterans. Other parenchymal lesions were mild interstitial fibrosis in 12, alveolar hemosiderosis and alveolar overinflation in 10 each, and alveolar septal calcinosis with chronic renal insufficiency in 3. Pleural effusion, pleuritis, or pleural thickening were noted in 15 of 26, 6 of 23, and 7 of 23 evaluable patients, respectively. Vascular lesions were present in 16 as intimal thickening (9), thromboemboli (8), medial hypertrophy (6), calcinosis (3), and vasculitis (2). A previously unreported lesion was chronic (proliferative) peribronchitis, noted in 11 patients. Diaphragmatic lesions included mild variation in fiber size in 7, mild fibrosis in 2, and calcinosis in 1 of 13 evaluable patients. Correlation of the above lesions with previously described lung syndromes in SLE such as lupus pneumonitis, hemorrhagic lung disease, chronic interstitial fibrosis, lupus cor pulmonale, pleurisy, and "shrinking lung syndrome" are discussed.
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PMID:Pulmonary lesions in childhood onset systemic lupus erythematosus: analysis of 26 cases, and summary of literature. 360 12

We describe four patients aged 14 to 21 years who developed acute aortic dissection. In three of the four patients, the course was fatal, despite aggressive medical and surgical intervention. All four patients had sustained systemic hypertension related to chronic renal insufficiency. The patients had no other identifiable risk factors for aortic dissection, including congenital cardiovascular disease, advanced atherosclerosis, vasculitis, trauma, pregnancy, or family history of aortic dissection. Although aortic dissection is rare in individuals younger than 40 years of age, young patients with sustained systemic hypertension are at increased risk for this serious and often fatal condition. Physicians must be aware of this rare complication of hypertension and consider aortic dissection in the differential diagnosis of unusual chest, abdominal, and back pain in hypertensive children, adolescents, and young adults.
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PMID:Aortic dissection in young patients with chronic hypertension. 1002 53

Acute Renal Failure (ARF) is characterized by a rapid decline of the glomerular filtration rate, due to hypotension (prerenal ARF), obstruction of the urinary tract (post-renal ARF) or renal parenchymal disease (renal ARF). The differential diagnosis among different causes of ARF is based on anamnesis, clinical symptoms and laboratory data. Usually ultrasound (US) is the only imaging examination performed in these patients, because it is safe and readily available. In patients with ARF gray scale US is usually performed to rule out obstruction since it is highly sensitive to recognize hydronephrosis. Patients with renal ARF have no specific changes in renal morphology. The size of the kidneys is usually normal or increased, with smooth margins. Detection of small kidneys suggests underlying chronic renal pathology and worse prognosis. Echogenicity and parenchymal thickness are usually normal, but in some cases there are hyperechogenic kidneys, increased parenchymal thickness and increased cortico-medullary differentiation. Evaluation of renal vasculature with pulsed Doppler US is useful in the differential diagnosis between prerenal ARF and acute tubular necrosis (ATN), and in the diagnosis of renal obstruction. Latest generation US apparatus allow color Doppler and power Doppler evaluation of renal vasculature up to the interlobular vessels. A significant, but non specific, reduction in renal perfusion is usually appreciable in the patients with ARF. There are renal pathologic conditions presenting with ARF in which color Doppler US provides more specific morphologic and functional information. In particular, color Doppler US often provides direct or indirect signs which can lead to the right diagnosis in old patients with chronic renal insufficiency complicated with ARF, in patients with acute pyelonephritis, hepatic disease, vasculitis, thrombotic microangiopathies, and in patients with acute thrombosis of the renal artery and vein. Contrast enhanced US is another useful diagnostic tool in patients with ARF which has been recently introduced in clinical practice. Microbubble administration may reduce technical failure in the evaluation of the renal artery. Moreover, perfusion defects due to stenosis or thrombosis of the renal segmentary vessels are better recognized. New diagnostic possibilities of enhanced US include evaluation of both cortical and medullar vessels, and functional evaluation of renal perfusion. Measuring the transit time of the microbubbles is useful for the diagnosis of renal artery stenosis and, in transplanted kidneys, for differential diagnosis between ATN and acute rejection.
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PMID:[Current role of color Doppler ultrasound in acute renal failure]. 1177 81

Mixed cryoglobulinemia (MC) and glomerulonephritis are the most important extrahepatic manifestations of chronic hepatitis C virus (HCV) infection. MC is a non-neoplastic B cell lymphoproliferative process induced by HCV in an antigen-driven mechanism. The clinical expression of cryoglobulinemia varies from an indolent course to the development of systemic vasculitis. Glomerulonephritis is predominantly associated with MC, and almost always takes the form of membranoproliferative glomerulonephritis. The renal manifestations may range from isolated proteinuria to overt nephritic or nephrotic syndrome with variable progression towards chronic renal insufficiency. The treatment of these virus-related diseases must be individualized on the basis of the severity of clinical symptoms. Antiviral therapy with interferon alpha and ribavirin (the currently recommended treatment of HCV infection) may be successful in patients with mild-to-moderate disease, but sustained responses are uncommon. In case of severe and rapidly progressive disease, although it is capable of suppressing viremia and cryoglobulinemia, antiviral therapy is not fully effective in controlling the inflammatory and self-perpetuating reaction consequent to the deposition of cryoglobulins in the glomeruli and vessel walls. In such cases, a short course of steroids and cytotoxic drugs (with or without plasmapheresis) may be needed to improve the vascular manifestations and decrease the production of cryoglobulins. Once the acute disease flare has been controlled, antiviral therapy may be administered to eradicate HCV, the causative agent of the cryoglobulinemic syndrome. In patients in whom antiviral therapy is ineffective, contraindicated or not tolerated, rituximab, a monoclonal anti-CD20 antibody, may be an alternative to standard immunosuppression.
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PMID:Hepatitis C virus-related cryoglobulinemia and glomerulonephritis: pathogenesis and therapeutic strategies. 1605 39

Mixed cryoglobulinemia (MC) and glomerulonephritis are the most important extrahepatic manifestations of chronic hepatitis C virus (HCV) infection. The clinical expression of cryoglobulinemia varies from an indolent course to the development of systemic vasculitis. The renal manifestations may range from isolated proteinuria to overt nephritic or nephrotic syndrome with variable progression towards chronic renal insufficiency. Plasmapheresis appears to be a useful adjunct to conventional therapy in the treatment of anti-GBM nephritis, severe dialysis-dependent forms of pauciimmune RPGN, cryoglobulinemia, and HUS-TTR Therapy with plasmapheresis produced a marked decrease in cryoglobulin levels and a subsequent relevant clinical improvement of cutaneous lesions and renal function. In cryoglobulinemia, plasmapheresis can be used as effective further therapy to minimize cutaneous, renal and/or neurologic involvement.
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PMID:Plasmapheresis in cryoglobulinemic neuropathy: a clinical study. 1793 17

Calcinosis has long been associated with autoimmune disease and has a distinctive profile in scleroderma, dermatomyositis, systemic lupus erythematosus, and overlap syndromes. However, there have also been a number of case studies of calcific uremic arteriolopathy, or calciphylaxis, described within vessels, including patients with chronic renal insufficiency and several forms of vasculitis. Interestingly, the calciphylaxis associated with vasculitis appears to be unique, although relatively uncommon and is likely secondary to a disruption in the calcium-phosphate-parathyroid hormone axis. However, there appears to be an additional trigger, given that calciphylaxis is seen both in the absence of chronic kidney disease, and in the absence of a deranged calcium-phosphate-parathyroid hormone axis. These additional triggers include a high female predominance, obesity, diabetes and, possibly, warfarin use. In this review, we describe the clinical features of calciphylaxis, particularly in the context of autoimmune disease.
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PMID:Recognizing calcific uremic arteriolopathy in autoimmune disease: an emerging mimicker of vasculitis. 1877 19

Renal activity and smoldering disease is difficult to assess in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) because of renal scarring. Even repeated biopsies suffer from sampling errors in this focal disease especially in patients with chronic renal insufficiency. We applied capillary electrophoresis coupled to mass spectrometry toward urine samples from patients with active renal AAV to identify and validate urinary biomarkers that enable differential diagnosis of disease and assessment of disease activity. The data were compared with healthy individuals, patients with other renal and non-renal diseases, and patients with AAV in remission. 113 potential biomarkers were identified that differed significantly between active renal AAV and healthy individuals and patients with other chronic renal diseases. Of these, 58 could be sequenced. Sensitivity and specificity of models based on 18 sequenced biomarkers were validated using blinded urine samples of 40 patients with different renal diseases. Discrimination of AAV from other renal diseases in blinded samples was possible with 90% sensitivity and 86.7-90% specificity depending on the model. 10 patients with active AAV were followed for 6 months after initiation of treatment. Immunosuppressive therapy led to a change of the proteome toward "remission." 47 biomarkers could be sequenced that underwent significant changes during therapy together with regression of clinical symptoms, normalization of C-reactive protein, and improvement of renal function. Proteomics analysis with capillary electrophoresis-MS represents a promising tool for fast identification of patients with active AAV, indication of renal relapses, and monitoring for ongoing active renal disease and remission without renal biopsy.
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PMID:Identification and validation of urinary biomarkers for differential diagnosis and evaluation of therapeutic intervention in anti-neutrophil cytoplasmic antibody-associated vasculitis. 1956 50