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)

Vasculitis associated with hairy-cell leukemia (HCL) has been reported occasionally. We determined the clinical and biological significance of this association by the retrospective study of a series of 50 patients with HCL, including nine patients with vasculitis. The development of vasculitis was not related to a variant of HCL on the basis of hematologic findings and survival. Vasculitis could occur at any time during the course of HCL, and was the circumstance for the diagnosis of HCL in three cases. Clinical and immunohistologic findings were those of hypersensitivity vasculitis in the nine patients. Infection was found to be an associated factor. Thus, eight of nine patients were infected at vasculitis onset, four died of their infection with no remittance of the cutaneous lesions, and three recovered from both infection and vasculitis. The monocyte deficiency in HCL is known to favor intracellular pathogen infection; however, we could not demonstrate that it also correlates with a decrease in the clearance of IgG-sensitized erythrocytes. Finally, vasculitis in HCL appears to be associated with a lasting infection in most cases.
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PMID:Vasculitis in hairy-cell leukemia. 382 53

Newborn cats, pigs, and sheep (3 to 14 days old) and postweanling cats (2.5 months old) that had been inoculated with Gardner feline sarcoma virus and feline leukemia virus at 10 days of age were infected experimentally with a rabbit isolate of the mammalian protozoan parasite Encephalitozoon cuniculi. Infection occurred in all cats and in some sheep, but was questionable in pigs. Brain and kidney were the 2 major target organs in cats. The lesions were compatible with, but less severe than, those of naturally infected cats and other carnivores. Of 13 cats, E cuniculi could be detected morphologically in the kidneys of 12 cats and in the brain of 1 cat. The organisms were reisolated from 2 cats with ground tissue suspension of kidney or urine sediment. The indirect immunofluorescence antibody (IFA) titers were 1:20 to 1:1,280 at the time the animals were killed, but antibodies were not detected before inoculation. Lesions were seen in the kidneys of 2 of 4 sheep. These lesions were mild, but were compatible with those in a spontaneously affected goat. Encephalitozoon cuniculi were found morphologically in the kidney of 1 sheep with lesions. All sheep had IFA titers of 1:10 to 1:20 before inoculation, and the titers were 1:20 to 1:320 when they were killed. Vasculitis, similar to the subacute-to-chronic stage of polyarteritis nodosa, was observed in 1 of 8 pigs. The lesions were primarily present in the kidney; comparable but milder lesions were also seen in the heart and brain. Antibody was not detected before inoculation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Susceptibility of cats, sheep, and swine to a rabbit isolate of Encephalitozoon cuniculi. 392 75

Vasculitis may give rise to different clinical symptoms according to the vessels involved, but its pathology is always the same: inflammation and, sometimes, necrosis of blood vessels. Most vasculitis are associated with immune mechanisms, notably formation of immune complexes. Infections, drug reactions and systemic or malignant diseases are sometimes accompanied with vasculitis, but they do not confer specificity to the anatomo-clinical picture. A classification of the various types of vasculitis including these factors provides a more rational approach to therapy. It must be remembered, in particular, that corticosteroids and cytotoxic agents, already used successfully in Wegener's granulomatosis, have a place in the treatment of other forms of systemic necrotizing vasculitis.
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PMID:[Vasculitis. Immunological, anatomo-clinical and therapeutic study (author's transl)]. 611 60

Patients with HCL are subject to a variety of medical problems. Many of these complications are caused by the cytopenias and splenomegaly produced by proliferating neoplastic cells. Infection is a common cause of morbidity in HCL, but it is not clear whether there is an inherent defect in the immune system. The incidence of infection is related to neutropenia and is increased by the administration of cytotoxic drugs and corticosteroids; such drugs should be used cautiously in these patients. Opportunistic or unusual pathogens occur frequently in HCL, but recovery from such infections is the rule if the diagnosis is made early. Marrow hypoplasia is not infrequently seen and may present diagnostic difficulties. Such patients may have a lower tumor burden and clinically milder anemia. Hemorrhagic complications are unusual in HCL, though many patients have platelet function abnormalities. Other medical problems occur with increased frequency in HCL, and failure to recognize them leads to increased morbidity in this disease. Autoimmune disease is seen in up to one fourth of patients. It takes the form of self-limited skin and joint disease, or a more progressive, systemic of patients. It takes the form of self-limited skin and joint disease, or a more progressive, systemic vasculitis. Both forms can usually be treated with splenectomy or corticosteroids, but alkylating agents can also be used successfully. Bone disease is usually localized and responds well to radiotherapy. Other problems such as amyloidosis, multiple myeloma, and paraproteinemia are uncommon in HCL.
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PMID:Clinical problems in hairy cell leukemia: diagnosis and management. 639 Jun 85

The case is presented of a premature infant with Pseudomonas aeruginosa infection, apparently acquired in utero. After a complicated postnatal course, the child was noted to have a profound hearing loss. This infection itself, was rapid and progressive, with the infant showing signs and symptoms characteristic of Pseudomonas infection, such as necrotizing skin vasculitis and "green" purulent discharge. Pseudomonas infection poses a virulent and life-threatening challenge to the immunologically immature infant. Infection with this organism, uncommon in the neonate, results in significant morbidity and mortality.
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PMID:Intrauterinely acquired Pseudomonas infection in the neonate. 681 12

Tissues from corticosteroid-treated gerbils hyperinfected with Strongyloides stercoralis were compared grossly and microscopically to similar tissues from animals with uncomplicated strongyloidiasis. Gerbils with hyperinfection developed severe pulmonary alveolar haemorrhage with a variable degree of subacute eosinophilic interstitial pneumonia associated with numerous alveolar, vascular and interstitial larvae. Hyperinfection induced by corticosteroids, given either before inoculation of S. stercoralis larvae or after a chronic Strongyloides infection was established, produced similar lesions. In contrast, lungs from gerbils with uncomplicated Strongyloides infection had severe eosinophilic perivasculitis and vasculitis with very little haemorrhage, no pneumonia and no larvae. Sections of adult worms were present in the proximal part of the intestinal tract, lodged in spaces between mucosal epithelial cells. Adult worms were not associated with inflammation and were more common in the corticosteroid-treated gerbils. In corticosteroid-treated gerbils only, there were numerous larvae in the distal intestinal tract, throughout the intestinal wall and adjacent mesentery, within interstitial tissues and in lymphatic vessels. Significant inflammation with associated larvae was only present in the caecum and mesenteric lymph nodes, suggesting that the caecum was the main site for initiation of parenteral migration with subsequent invasion of the lymphatic system and lungs. The lesions in these gerbils were similar to those found in humans. Infection of gerbils with S. stercoralis is the best rodent model of human strongyloidiasis.
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PMID:Strongyloides stercoralis: histopathology of uncomplicated and hyperinfective strongyloidiasis in the Mongolian gerbil, a rodent model for human strongyloidiasis [corrected]. 763 16

This study was designed to investigate serum soluble interleukin-2 receptor (S-IL-2R), interleukin-2 (IL-2) and interleukin-6 levels (IL-6) in patients with either a positive or negative Borrelia burgdorferi serology. Serum samples from 101 individuals, divided in to five groups according to clinical symptoms and outcome of serology were analysed. Samples of cerebrospinal fluid (CSF) from nine of the individuals were also studied. The highest average serum S-IL-2R levels (1,180 +/- 1,140 U/ml) were found in patients with erythema migrans, the hallmark of Lyme borreliosis, followed by patients with symptoms closely related to Borrelia infection (900 +/- 1,200 U/ml) and with a strong positive serology. In two patients with central nervous system (CNS) involvement, increased levels of S-IL-2R of 920 and 620 U/ml respectively (normal value < 50 U/ml) were detected in the CSF. No statistically significant relationship between IgG or IgM antibody activity and serum S-IL-2R levels was found. Detectable levels of IL-2 were only found in three patients. Increased levels of IL-6 were found in sera from 14 patients. The highest concentration, 90 pg/ml (normal value < 10 pg/ml), was measured in a patient presenting with vasculitis. In conclusion, B. burgdorferi infection causes a moderate increase of serum S-IL-2R levels, although there is no relationship between the severity of the infection, as estimated by the antibody concentration or to serum IL-2 or IL-6 levels. Secondary complications of the infection, such as vasculitis, may cause an increased level of serum IL-6.(ABSTRACT TRUNCATED AT 250 WORDS)
Infection
PMID:Response of soluble IL-2 receptor, interleukin-2 and interleukin-6 in patients with positive and negative Borrelia burgdorferi serology. 784 8

A case of polyarteritis nodosa identified by the American College of Rheumatology (ACR) 1990 criteria in a 44-year-old HIV-infected man is described. The search for cytomegalovirus, HBV and B19 parvovirus infections was negative. In situ hybridization did not reveal proviral HIV-1 DNA in a skin sample. A zidovudine-associated vasculitis was excluded. Corticosteroid therapy resolved vasculitis manifestations and was well tolerated without opportunistic infections during the 10-month follow-up period. An indirect pathogenetic role of HIV as a possible cause of vascular damage cannot be excluded in our patient.
Infection
PMID:Polyarteritis nodosa and HIV infection: no evidence of a direct pathogenic role of HIV. 874 Jan 12

Hepatitis C is becoming the main cause of cirrhosis and primary liver carcinoma. Infection by hepatitis C virus (HCV) generally induces an asymptomatic acute hepatitis. HCV infection becomes chronic in about 80% of cases. In a minority of the subjects, chronic HCV infection is asymptomatic with persistent viremia and normal liver tests. These asymptomatic subjects have minimal liver histologic lesions and a good prognosis. In a majority of the subjects, chronic HCV infection is associated with chronic hepatitis with increased serum transaminases levels. Among the patients with chronic hepatitis, the majority have a mild liver disease with a moderate increase in serum transaminases levels and, at liver histology, minimal lesions; a minority (about 20%) have a more severe liver disease and will develop cirrhosis after 5 to 20 years. In patients with HCV related cirrhosis, the incidence of hepatocellular carcinoma is high (around 5% per year). The factors influencing the evolution of HCV infection are not known. Alcohol is certainly an important factor which increases the risk of development of fibrosis then cirrhosis. Virus related factors, such as genotype and level of replication, might also be important. Autoimmune diseases have been reported in association with hepatitis C. HCV infection is a major cause of mixed cryoglobulinemia associated with vasculitis or glomerulonephritis. A relationship between HCV and auto-immune diseases such as thyroiditis or Gougerot syndrome has been suggested but not demonstrated. HCV infection is frequent in patients with porphyria cutanea tarda; in these patients, HCV related liver disease might trigger the expression of the metabolic disease.
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PMID:[Clinical picture and evolution of hepatitis C]. 899 8

Infection with hepatitis C virus (HCV) may affect not only the liver but also various nonhepatic tissues and organs and may combine with many etiologically unrelated diseases and morbid conditions. Numerous nonhepatic manifestations in HCV infection have been previously reported. For some (eg, cryoglobulinemia), the association is well established. For others, such as sialadenitis and lichen planus, the association is probable (but not completely documented) and, for the remainder, the associations are weak. Extrahepatic manifestations may result from immunological mechanisms as well as virus invasion and replication in the affected extrahepatic tissues and organs. Thyroid abnormalities, primarily Hashimoto's disease, and isolated increases of anti-thyroid antibodies (ATPO) appear to be more frequent in chronic hepatitis C than B or D, with high ATPO titers clustering mainly among females. Interferon-alpha (IFN-alpha) therapy is associated with development of thyroid dysfunction in 5.5-12.9% of patients, usually exposing preexisting subclinical thyroid abnormalities. Mixed cryoglobulinemia (MC) is commonly found (36-45%) in patients with chronic HCV infection; however, only in a minority of cases does it become clinically manifested as systemic vasculitis with purpura, neuropathy, or Raynaud's phenomenon. In a number of patients, MC may terminate in non-Hodgkin's B-cell lymphoma. Treatment of these lymphoproliferative disorders with IFN-alpha is advocated. Idiopathic thrombocytopenia is now recognized more frequently in association with chronic HCV infection and is usually aggravated by IFN-alpha therapy. Patients with porphyria cutanea tarda (PCT) have demonstrated serological markers of HCV infection in 62-82% of cases. The usefulness of IFN-alpha in PCT remains to be demonstrated. Lichen planus has also been found in association with chronic HCV infection, particularly when severe or affecting the oral cavity. Other nonhepatic manifestations have also been reported in HCV infection such as diabetes, corneal ulceration, uveitis, and sialadenitis. These manifestations deserve further study and documentation. Finally, markers of autoimmunity occur with high frequency in chronic HCV infection; however, combination with the classical syndrome of autoimmune hepatitis is rare. In the presence of various autoantibodies, the clinical features of chronic hepatitis C do not appear to be modified and, contrary to general perception, IFN-alpha therapy within randomized controlled trials should not be withheld since the response rate to IFN-alpha does not appear to differ in the presence or absence of low titers of these markers.
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PMID:Nonhepatic manifestations and combined diseases in HCV infection. 901 79


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