Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We sought to explore immunological factors in patients who died with rapidly fatal fibrosing lung diseases (Hamman-Rich syndrome). A retrospective review of cases of interstitial lung disease showed 12 recent deaths from Hamman-Rich syndrome. The mean age was 62, men outnumbering women 3 : 1. Five patients had proved collagen vascular disease (rheumatoid arthritis three, lupus two). Four others had a history of allergic disorders, syphilis, chronic eosinophilia, or hypersensitivity reactions. One patient showed disappearance of immunofluorescence as fibrosis advanced, which has not previously been reported. The study suggests a possible aetiological link between disorders of immunity and Hamman-Rich syndrome. The evidence also supports the notion that Hamman-Rich syndrome is an accelerated variant of the more indolent interstitial pneumonias.
 ...
PMID:Rapidly fatal pulmonary fibrosis: the accelerated variant of interstitial pneumonitis. 16 92

A profound defect in granulocyte chemotaxis was documented in an otherwise healthy 21-yr-old man who failed to localize granulocytes to an area of cellulitis during an allergic reaction to cephalothin. During the period of drug allergy, characterized by urticaria, eosinophilia, and profound hypocomplementemia, in vitro migration of the patient's granulocytes in the Boyden chamber was markedly impaired. Although devoid of hemolytic complement activity, the patient's serum possessed supranormal chemotactic activity, even following heat inactivation, suggesting the presence of chemotactically active complement split products. Chemotactic function improved concomitantly with steroid therapy and normalization of serum complement levels, and was entirely normal following clinical recovery and cessation of steroid therapy. The chemotactic abnormality noted in the patient's cells was reproduced in normal granulocytes by preincubation either with patient serum or with cobra venom-activated fresh (but not heated) normal serum, suggesting that in vivo exposure of granulocytes to activated complement was responsible for the patient's abnormal chemotactic response. This mechanism may contribute to the increased infection propensity noted in other conditions characterized by in vivo complement activation, such as rheumatoid arthritis and systemic lupus erythematosis.
 ...
PMID:Acquired granulocyte abnormality during drug allergic reactions: possible role of complement activation. 83 Mar 75

A child is reported with chorea as the initial presentation of acute lymphoblastic leukemia. Subsequent laboratory studies revealed marked eosinophilia and a lupus anticoagulant. No peripheral or central nervous system lymphoblasts were observed. The chorea, eosinophilia, and lupus anticoagulant all resolved once remission of the acute lymphoblastic leukemia was induced. It is suggested that acute lymphoblastic leukemia be included in the differential diagnosis of chorea and eosinophilia in childhood.
 ...
PMID:Chorea, eosinophilia, and lupus anticoagulant associated with acute lymphoblastic leukemia. 147 78

The iatrogenic L-tryptophan-induced eosinophilia-myalgia syndrome, often considered to be a "new" disease, has proven to be a remarkable mimic of the classic sclerosing rheumatologic disorders. Although subacute cutaneous lupus erythematosus remains a clinically defined entity, supportive histologic and immunopathologic findings have recently been proposed. Rheumatoid neutrophilic dermatitis needs to be added to our usual differential diagnosis of a neutrophilic dermatosis without leukocytoclastic vasculitis. The antiphospholipid syndrome is associated with noninflammatory vascular thrombosis and often has recognizable cutaneous findings. Finally, ANCA are a valuable adjunct in the systemic evaluation of patients with vasculitis syndromes and suggest a common pathogenesis for several of the systemic vasculitides.
 ...
PMID:Collagen vascular disease. 173 Jan 64

The extent of eosinophils in skin biopsy and peripheral blood specimens of patients with lupus panniculitis and morphea profunda was determined by studying 37 biopsies from 33 patients with lupus panniculitis and 55 biopsies from 53 patients with morphea profunda. Specimens from 8 (24%) of 33 patients with lupus panniculitis showed eosinophils, and 13 (25%) of 53 patients with morphea profunda had eosinophils in the subcutaneous tissue. In all cases, the diagnosis of lupus panniculitis or morphea profunda was established on the basis of other, more characteristic histologic features; the presence of eosinophils was incidental and not a diagnostic criterion. Occasional cases of lupus panniculitis or morphea profunda had numerous eosinophils. However, in most of the specimens, eosinophils were absent or observed in small numbers. Only 3% of patients with lupus panniculitis showed an increase in the number of eosinophils in the peripheral blood, whereas 47% of patients with morphea profunda had peripheral eosinophilia. The degree of hyaline necrosis in lupus panniculitis or of sclerosis in morphea profunda did not correlate with the number of eosinophils present in the biopsy specimen or peripheral blood.
 ...
PMID:Eosinophils in lupus panniculitis and morphea profunda. 191 6

Early in the course of studies of the Spanish toxic oil syndrome it was recognized that vascular lesions were a major problem, most logically attributable to endothelial damage by the toxic oil. However, most clinical attention has been directed to the pulmonary complications and the evolution into a scleroderma-like illness later. In this study of 11 victims of the toxic oil syndrome careful postmortem studies of the coronary arteries and conduction system and neural structures of the heart demonstrated major injury to all those components of the heart. Obliterative fibrosis of the sinus node in four cases resembled findings in fatal scleroderma heart disease, and in eight the cardiac lesions resembled those of lupus erythematosus. The more impressive pathologic features involved the coronary arteries and neural structures, which were abnormal in every heart. The arterial disease included widespread focal fibromuscular dysplasia, but there was also an unusual myointimal proliferative degeneration of both small and large coronary arteries in five patients, four of whom were young women. In two hearts, portions of the inner wall of the sinus node artery had actually detached and embolized downstream. Coronary arteritis was rarely found. Inflammatory and noninflammatory degeneration of cardiac nerves was widespread. Fatty infiltration, fibrosis and degeneration were present in the coronary chemoreceptor. In most respects these cardiac abnormalities resemble those described in the eosinophilia-myalgia syndrome caused by an altered form of L-tryptophan. In both diseases there is good reason to anticipate more clinical cardiac difficulties than have so far been reported, and even more basis for future concern, especially relative to coronary disease and cardiac electrical instability.
 ...
PMID:Cardiac abnormalities in the toxic oil syndrome, with comparative observations on the eosinophilia-myalgia syndrome. 191 15

We experienced a case of idiopathic hypereosinophilic syndrome (HES) associated with pulmonary infarction. The case was a 22-year-old woman with marked eosinophilia (16835/microliters) and peripheral edema and cyanosis. During hospitalization, she suddenly developed lower right chest pain, and infiltrative lesions with pleural effusions in the right lung were prominent. A diagnosis of pulmonary infarction was made after perfusion scan and angiography of the lung. Lupus anticoagulant was found to be positive and a transient increase of anti-cardiolipin antibody slightly in her serum was also observed. Recurrent thrombosis is known to be complicated by HES but its mechanism remains to be clarified. There has also been no study reported in the literature on the role of lupus anticoagulant in this process; its possible role in this patient is discussed.
 ...
PMID:[A case of idiopathic hypereosinophilic syndrome (HES) complicated by pulmonary infarction]. 195 37

An unusual case of angiolymphoid hyperplasia with eosinophilia (ALHE) simulating port-wine stain in a 50-year-old woman is reported. The lesions of ALHE are typically papules or subcutaneous masses that range from light pink to red-brown in color. In addition to the usual histologic findings of ALHE, the biopsy in our patient showed some fibrin-like material and fibrous long-spacing collagen on ultrastructural examination. This unusual lesion necessitates biopsy because the differential diagnosis includes port-wine stain, sarcoidosis, lupus erythematosus, and non-Hodgkin lymphoma (mycosis fungoides). Many different forms of treatment have been attempted for ALHE including radiotherapy, cytotoxic chemotherapy, corticosteroids, and antibiotics. The lesions in our patient responded to argon laser therapy and surgical excision, though there has been recurrence on the border of the treated area. Because laser energy is noncumulative in the tissues and effective in removing the lesions, we recommend it as the treatment of choice for these lesions.
 ...
PMID:Angiolymphoid hyperplasia with eosinophilia-acquired port-wine-stain-like lesions: attempt at treatment with the argon laser. 323 58

Fifty randomly selected asthmatic patients were studied. In each the sex, age, time of onset of symptomatology, symptomatologic score (from 0 to 3), blood eosinophilia, IgE by the PRIST method and Tart cells in blood (investigated by the same method as is used in the search of LE cells in collagen diseases) were considered. Tart cells were recognized as in Miale Haemotology (9) as monocytes and occasionally polymophonuclar leucocytes with one or two round inclusions in their protoplasm owing to the phagocytosis of leukocyte nuclei. In contrast with LE cells, these inclusions are not homogeneous contain chromatin and nuclear membrane material and often have a dark ring of hyperchromic material. We have found that these cells can not be evoked passively by application of patient serum to normal leukocytes. In no case of positive Tart cells was antinuclear serum factor found by immunofluorescence as seen in lupus erythematous disease. 33 per cent of the 50 asthmatic patients tested had Tart cells in their blood. They were very scarce, only one or two in four Wright stained smears. This fact is probably why these cells were not recognized before. The positive Tart cell group was on the average the same age as the negative group. On the other hand, in the positive Tart cell group the proportion of men with respect to women, the time span from the onset of asthmatic symptoms (p less than 0,05), the symptomatology score (p less than 0,005) and blood eosinophilia (p less than 0,005) were increased. In contrast IgE was decreased on the average (p less than 0,05).(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Finding of Tart cells in asthmatic patients. Relation to IgE and other parameters]. 620 75

Interferon alpha (INF-alpha)--In systemic diseases, most indications for INF-alpha result from its effect on haematological or hepatological manifestations. The spectacular effect of INF-alpha in chronic myeloid leukemia has led to its use for the treatment of hypereosinophilia syndrome and systemic mastocytosis. Over the last 6 years, we have treated 7 patients with the hypereosinophilia syndrome who were resistant to corticotherapy and had markers of myeloproliferation. Although both hydroxyurea and INF-alpha can be effective alone, their combination led to a decrease in the eosinophilia count to 1,000/ml, a decrease which was long-lasting in most cases. INF-alpha is also used in histiocytosis X alone or in combination with retinoids or with etoposide and has been found effective in several observations. In carcinoid syndromes whether treated priorly or not with a 5-fluoro-uracil-streptozoticin combination, INF-alpha leads to an objective response in two-thirds of the patients. Several multicentric protocols are currently assessing the efficacity of INF-alpha in mixed cryoglobulinaemias. In most observations these cryoglobulinaemias are seen in patients with markers of hepatitis C (mainly HCV) and the early results are encouraging. Temporary improvement has been reported in discoid or subacute lupus in 8 out of 10 cases. Haemangiomas of the infant, when life-threatening and corticoresistant, may be a good indication for INF-alpha. Thus 20 newborns or infants (including 4 with Kasabach-Merrit syndrome) have been treated with good results in 18. Interferon gamma (INF-gamma).(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Interferons. Interferons alpha and gamma: indications in systemic diseases]. 817 44


1 2 3 Next >>