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)

Subacute meningitis was the first manifestation of illness in a young man with multisystem disease involving lung, kidney, eye, and central nervous system. Necrotizing granulomatous angiitis was found in tissue obtained at thoracotomy and the diagnosis of Wegener's granulomatosis was suggested. This patient had an excellent functional recovery with cyclophosphamide and prednisone.
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PMID:Subacute meningitis heralding a diffuse granulomatous angiitis: (Wegener's granulomatosis?). 55 62

To determine the significance of various histopathologic features of pancreatic rejection, we reviewed the pathology of 53 biopsies taken to rule out rejection [32 bladder drained, (BD); 18 non-BD]. Twenty-six biopsies from 23 patients with allografts which ultimately failed (FLD) (7 BD, 16 non-BD) were compared with 27 biopsies from 27 patients with allografts which continue to function (FXN) (25 BD, 2 non-BD). The groups are similar in regard to age, sex, and time after transplant to biopsy. The mean follow-up is 13 months for FLD grafts versus 35 months for FXN grafts (p < 0.0001). In BD grafts, decreases in urine amylase usually led to biopsy, while in non-BD grafts, hyperglycemia usually prompted biopsy. More patients with ultimately FLD organs (17 of 26) presented with elevated blood glucose (BG) than patients with FXN grafts (2 of 27) (p < 0.0001). Multiple histologic features were examined related to the acinar tissue, pancreatic ducts, islets, vessels, and nerves. Features which strongly correlated with a negative outcome included moderate to severe inflammation of acinar tissue (p < 0.0001), acinar tissue loss and fibrosis (p < 0.0087) and vascular luminal narrowing due to chronic rejection (p < 0.003). Twenty-one pancreases showed chronic rejection and were treated with OKT3 or anti-lymphocytic globulin (ALG), six of these continue to function 1.6-9 years after biopsy, including two who presented with elevated BG levels. A normal biopsy was found in nine pancreases, all of which continue to function. Vasculitis was only seen in biopsies with moderate to severe inflammation, whereas endothelialitis was also seen in association with mild inflammation, suggesting that vasculitis is a more aggressive lesion. A rejection classification is proposed with endothelialitis partly defining mild rejection and vasculitis defining severe rejection. We conclude that several biopsy features and elevation of BG are strongly correlated with a high probability of failure; however, antirejection therapy is justified because recovery of function occurs in some cases. A normal biopsy obviates the need for therapy and predicts a good outcome, as do mild histological findings of rejection.
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PMID:Pancreas rejection. Significance of histopathologic findings with implications for classification of rejection. 147 30

The medical records of 47 children with dermatomyositis who were seen in the pediatric rheumatology clinic at the University of Michigan between 1964 and 1982 were reviewed. Although most children with dermatomyositis have a good prognosis, the best predictor of both good functional recovery and minimal calcinosis is early treatment after the onset of symptoms, using high doses of prednisone for an adequate length of time. Of the children given such treatment, 78% had good functional outcomes, and disabling calcinosis was seen in 20% or less. Children given treatment late in the course of disease and with low doses of steroids are more likely to be functionally limited and have a greater amount of dystrophic calcium salt deposition. In our study, only 33% of patients given such treatment had a mild disease course with good functional outcome. We have identified a subgroup of children with dermatomyositis who appear to do poorly despite optimal therapeutic regimens. These patients are distinguished by a severe disease course responding minimally to corticosteroid therapy and manifested by persistent muscle weakness, elevations of muscle enzyme activity, and severe generalized cutaneous vasculitis. These children are at high risk for the development of exoskeleton-like calcification; consideration should be given to combined immunosuppressive therapy early in the course of disease.
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PMID:Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification. 664 23

Spontaneous spinal epidural haematoma can occur in the elderly and is a reversible cause of neurological deficit if treated promptly. The diagnosis can be made from a careful history and a simple neurological examination, but it can be confused with myocardial infarct, musculoskeletal pain, vasculitis and acute dissection of an aortic aneurysm. For a favourable outcome, early decompressive laminectomy and evacuation of the haematoma are necessary. We report an unusual case of acute quadriplegia in which prompt diagnosis and early intervention led to almost complete functional recovery with minimal disability.
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PMID:Spontaneous cervical epidural haematoma. 953 93

The incidence of vascular complications due to drug abuse is at present increasing due to new types of drugs and to the different ways of intake of such substances. The vascular complications related to drug abuse may affect venous, arterious and lymphatic districts and in particular: ischemia following intra-arterial injections, arterious and venous pseudoaneurysm, vasculitis, aneurysms, aortic dissections, abscesses complicated by erosions of vessels, arteriovenous fistulas, compartment syndrome, superficial and deep venous thrombosis, septic trombophlebitis, puffy hand syndrome. The scientific knowledge in this matter is incomplete because of the new pathological cases and the lack of information regarding the efficacy of different treatments. The authors report four patients affected by vascular pathologies due to drug abuse. In one case, a heroin addict has undergone multiple fasciotomies for compartimental syndrome arising because the patient maintained an innatural posture for several hours during an overdose coma. In a second case, a segmental right subclavear deep venous thrombosis has been treated by pharmacological therapy with satisfactory functional recovery of the arm. A third patient has been successfully submitted to intra-arterial pharmacological vasodilatation for generalised lower limbs vasospasm caused by drug abuse. In the last case, the voluntary swallowing of a great dose of cocaine caused the patient's death after multiple ischemic and hemorrhagic cerebral episodes. After the description of these cases, a review of the recent literature and some observations on this topic are presented. A better knowledge of vascular complications due to drug abuse should improve the therapeutical approach of these patients.
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PMID:[Vascular pathology of surgical interest in drug addicts]. 1119 58

The clinical, electrophysiological and pathological features and prognosis of 9 patients with nonsystemic vasculitic neuropathy are described. Nonsystemic vasculitic neuropathy accounted for 3% of cases of biopsy proven cases of various neuropathies and formed 56% of vasculitic neuropathy. Both clinically and on electrophysiological testing, mononeuritis multiplex was the form of neuropathy in 5 patients and 3 had sensory neuropathy. All the patients had a necrotizing vasculitis on nerve biopsy. Axonal degeneration was seen in teased fibers in all the patients. Eight patients showed good functional recovery one was left with mild bilateral claw hands.
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PMID:Nonsystemic vasculitic neuropathy. 1127 13

Systemic lupus erythematosus is a multisystem disease with a large spectrum of clinical manifestations and a variable course. Lupus is marked by both humoral and cellular immunologic abnormalities, including multiple auto-antibodies especially anti DNA antibodies. Epidemiology - female predominance, occurring usually between second and fourth decade of life, more frequently in hispanic and black patients. Family predominance has been noticed. Provocative agents - ultraviolet light, viral infections, drugs and situational stresses. Pathogenesis - pathological features can affect a large spectrum of internal organs and systems - osteoarticulary injuries, skin rash, lymphadenopathy, glomerulonephritis, myocarditis, digestive system lesions. Musculo skeletal abnormalities include migratory arthritis, effusion and stiffness in small and large joints. Articular erosions are uncommon. Skeletal abnormalities include osteopenia and osteonecrosis, due to two pathological mechanisms: vasculitis and long term corticotherapy. Fifteen to twenty percent of SLE patients are affected by femoral head avascular necrosis (FHAN). Diagnosis rests on clinical signs - hip pain, limited range of motion, walking with a limp.; radiological findings - best grouped in Arlet-Ficat standing system; MRI - high sensitivity, especially in infraradiological stages. Treatment - in incipient stages core decompression represents the best therapeutical option. In advanced, arthritis stages, total hip arthroplasty (THA) is the standart treatment. Three implant types are available: bipolar, uncemented and cemented. An increased number of cotyloidites occurred after bipolar implants. Emphasised osteopenia and excessive bleeding represent contraindications for uncemented implants. Considering all of this, cemented implants are considered, the right choice, methacrylate cement providing strong and durable fixation of THA implants to bone. No meaningful differences were observed in postoperative functional recovery between LSE patients and other etiology FHAN patients.
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PMID:Total hip arthroplasty in secondary systemic lupus erythematosus femoral head avascular necrosis. 1796 53

Spontaneous intracranial artery dissection (SIAD), leading to occlusion or stenosis of arterial lumen is a frequent but less studied cause of ischemic stroke especially in young adults. We studied 17 patients (10 men, 7 women, mean age 27.5 +/- 8.5 years) with SIAD. All the patients have undergone magnetic resonance imaging of the head, magnetic resonance angiography (MRA) (in 16 patients--follow-up MRA), 1 patient--conventional cerebral angiography. SIAD was located in the middle cerebral artery (MCA, 14 patients); posterior cerebral artery (PCA, 2 patients) and basilar artery (BA, 1 patient). Fifteen patients (88%) with SIAD developed ischemic stroke, 2 patients (12%)--a transient ischemic attack (TIA). The appearance of the local brain ischemia symptoms was combined with headache in 93% cases. The course of ischemic stroke was favorable in most cases with complete or good functional recovery in 66% of patients. A fatal outcome was observed in 1 patient (7%) with massive brain infarct. The initial cerebral angiography carried out in most cases (76%) within 3 weeks after the disease onset revealed occlusion (71%) or stenosis (29%) of corresponding cerebral arteries (MCA, PCA, BA). The last cerebral angiography conducted in 90% cases 4,5 months or later showed positive dynamics--appearance or improvement of the blood flow in these arteries--in 82% patients. The factors provoking SIAD were alcohol, contraceptive drugs and less frequent recent infection. None of patients had atherosclerosis, vasculitis or arterial hypertension. Clinical manifestations of connective tissue weakness were in 71% of patients, hypotension--65%, mitral valve prolapse--46%. In conclusion, SIAD is one of the causes of ischemic stroke and TIA in young adults and characteristic clinical manifestations and follow-up MRA have a great diagnostic importance. The development of SIAD appears to be related to weakness of connective tissue of arterial wall.
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PMID:[Spontaneous intramural intracranial artery dissection and ischemic stroke]. 1819 22

Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are common in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lupus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases can be life-threatening if not treated promptly. Lupus mesenteric vasculitis is the most common cause, followed by protein-losing enteropathy, intestinal pseudo-obstruction, acute pancreatitis and other rare complications such as celiac disease, inflammatory bowel diseases, etc. No specific autoantibody is identified as being associated with SLE-related gastroenteropathy. Imaging studies, particularly abdominal computed tomography scans, are helpful in diagnosing some SLE-related gastroenteropathies. Most of these complications have good therapeutic responses to corticosteroids and immunosuppressive agents. Supportive measures such as bowel rest, nutritional support, antibiotics and prokinetic medications are helpful in facilitating functional recovery and improving the outcome.
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PMID:Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment. 2057 99

The association between Kaposi's sarcoma (KS) and human herpes virus eight (HHV-8) infection is rarely reported in hemodialysis (HD) patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV) infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.
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PMID:Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient. 2423 84


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