[Churg-Strauss syndrome. Seldom seen or only seldom diagnosed?].
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Diagnosis, Differential↗
Biomedical subjects
B J Manger
Publications and source records attributed to B J Manger.
[Total lymphoid irradiation in chronic polyarthritis--a new therapeutic concept].
Eleven patients with refractory rheumatoid arthritis were submitted to a total lymphoid irradiation up to a dose of 20 Gy. A constant improvement of clinical symptoms was observed in four out of the eleven patients already during the treatment and in the other patients not later than two months after. The frequency of attacks decreased and the number of joints involved in the attack was reduced. Morning rigidity and joint swellings decreased. One patient developed joint empyemas 4 and 26 months after the treatment. Four patients died in the meantime. In two patients the cause of death were renal insufficiency and a postoperative cardiogenic shock associated with generalized amyloidosis. The third patient died because of a toxically induced left cardiac decompensation with sepsis that could not be controlled by antibiotic drugs and multiple joint empyemas. The fourth patient developed an abscess after surgical treatment of a Kaposi syndrome. She died three months later from acute left cardiac decompensation. The therapy induced a lymphocytopenia with decrease of T helper lymphocytes and unchanged number of T suppressor lymphocytes. The constant therapy results of total lymphoid irradiation in primary chronic polyarthritis is probably due to this modification in the immune regulation.
Aged↗
Total lymphoid irradiation in patients with refractory rheumatoid arthritis.
Eleven patients with rheumatoid arthritis that had been refractory to conventional drug therapy were treated with total lymphoid irradiation (TLI). Followup continued for 6 months in 9 patients, 12 months in 6 patients, and 24 months in 3 patients. At 6 and 12 months post-TLI, a significant improvement in clinical disease activity was demonstrated. Side effects noted during TLI included fatigue, nausea, diarrhea, and vomiting. One patient died of cardiorespiratory arrest, 2 patients died of kidney failure secondary to generalized amyloidosis, and 1 patient died of septic shock secondary to a multilocular septic arthritis. One patient experienced 2 episodes of septic arthritis; 2 patients manifested delayed wound healing. Immunologic assessments showed consistent lymphopenia in all patients. T lymphocyte subsets decreased after TLI, and showed a transient increase at 6 months post-TLI. The suppressed mitogen responsiveness, which was noted 2 months after irradiation, was found to increase almost to the pre-TLI levels at 12 months. The observed increase in morbidity and mortality after TLI is evidence that discourages the use of this therapeutic technique, at least in its present form.
Antigen-Antibody Complex↗
The nonspecific clearance function of the reticuloendothelial system in patients with immune complex mediated diseases before and after therapeutic plasmapheresis.
The nonspecific clearance function of the reticuloendothelial system (RES) in six patients with immune complex mediated systemic vasculitis was determined by the evaluation of the disappearance rate of technetium 99m labelled microaggregated human serum albumin colloid (MHAC) injected IV before and after therapeutic plasma exchange. Three patients with systemic lupus erythematosus (SLE) and one patient with immune complex vasculitis (ICV) exhibited a significant clinical improvement after plasmapheresis which was paralleled by an accelerated MHAC elimination rate following plasma exchange therapy. One patient with ICV and unresponsive to plasma exchange showed delayed MHAC elimination. In one patient with myasthenia gravis (MG), the elimination rate was not altered by plasmapheresis. The data obtained indicate that nonspecific clearance of the RES may be one effect of plasma exchange therapy in patients with immune complex mediated diseases.
Adolescent↗
IgE-containing circulating immune complexes in Churg-Strauss vasculitis.
In five patients with vasculitis, hypereosinophilia, and elevated serum IgE levels a diagnosis of Churg-Strauss syndrome was established. To identify a possible role of IgE in pathogenic mechanisms leading to the vasculitis, we performed a sequential precipitation of the patients' sera with different concentrations of polyethylene glycol (PEG) 6000. Using a radio immunosorbent test, we tested the precipitates obtained for IgE. Considerable amounts of IgE were traced in the serum precipitates of all patients, especially after the second precipitation step (4.0% PEG). In contrast, no IgE-containing precipitates were detectable in sera from patients with different allergic diseases and high IgE serum levels. Together with an increase in C3d serum levels and the failure to demonstrate C1q-binding material in patients' sera, these data suggest the involvement of IgE-containing immune complexes in the pathogenesis of Churg-Strauss vasculitis, activating the complement via the alternate pathway.
Antigen-Antibody Complex↗
Immunological studies in homosexual men with unexplained lymphadenopathy.
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Homosexuality↗
[Effect of histamine-blocking substances on mitogen-induced lympho proliferation in rheumatic diseases].
Peripheral blood lymphocytes of patients with rheumatoid arthritis, psoriatic arthritis and healthy controls, isolated by a ficoll density gradient, were incubated with different concentrations of histamine, cimetidine and diphenhydramine (DPH) and simultaneously stimulated with phytohemagglutinine (PHA). In comparison to healthy control persons the lymphoproliferation rate in arthritic patients could be increased about 20% by addition of the histamine-blocking agents cimetidine and DPH. This suggests the existence of in vivo histamine-induced suppressor cells in arthritic patients, which can be inhibited by receptor blocking agents.
Arthritis, Rheumatoid↗