Search PubMed⌕ Search

Biomedical subjects

H Spiera

Publications and source records attributed to H Spiera.

At least 19 recordsLinked to original sources
PubMed1992

Systemic lupus erythematosus patients with central nervous system involvement show autoantibodies to a 50-kD neuronal membrane protein.

An antibody was detected in the sera from patients with systemic lupus erythematosus (SLE) and central nervous system (CNS) involvement that reacted with a 50-kD antigen in the plasma membrane of brain synaptic terminals. The 50-kD antigen was solubilized with Triton X-100 from preparations enriched with synaptic plasma membranes, and was partially purified by molecular sieve filtration column chromatography. The sera of 19 of 20 CNS-SLE patients showed strong to moderate immunoreactivity with the 50-kD protein in Western blots. Immunoreactivity with the 50-kD protein was also detected in the cerebrospinal fluid of CNS-SLE patients. Control sera from healthy individuals did not react with the 50-kD protein. Low to background reactivity was detected in 35% of a group of SLE patients without CNS manifestations, and in 3% of patients displaying other connective tissue diseases. A total of 100 individuals were tested in this study. Purified autoantibodies to the 50-kD protein from CNS-SLE patients were used for immunofluorescent labeling of neuroblastoma cells. The immunofluorescent staining revealed a distinct macular distribution pattern on the surface of the cell membrane. Taken together, the data suggest that the 50-kD protein may be an important target for autoantibodies, preponderantly found in CNS-SLE patients, and that the antigen may play a role in the pathogenesis of some neurological manifestations in SLE.

Adolescent↗
PubMed1992

Scleroderma in association with the use of bleomycin: a report of 3 cases.

Although an association between exposure to bleomycin and the development of scleroderma has been suspected, few cases are reported. We describe the development of cutaneous scleroderma in 3 patients coincident with the use of bleomycin in low cumulative doses of less than 100 U. Dramatic clinical improvement occurred in 2 patients treated with high dose steroids. Our series of 3 patients supports a causal connection between bleomycin and scleroderma. In light of the widespread use of bleomycin, this complication may be under-appreciated.

Adult↗
PubMed1992

The absence of factor B activation products in scleroderma.

Previous investigators have reported the presence of complement activation products in scleroderma. Using an assay consisting of isoelectric focussing followed by immunofixation (IEF/IF) for alternative pathway activation product Ba, and an ELISA for C3d that we developed for evaluating lupus plasmas, we assayed 48 plasmas from patients with diffuse cutaneous scleroderma, 16 patients with the limited CREST variant, 2 patients with mixed connective tissue disease (MCTD) and 4 patients with Raynaud's disease. Ba was not detected in any patient's plasma. Only one plasma from a patient with CREST contained elevated levels of C3d. We cannot reconcile the absence of B activation products in our patients with scleroderma with the results of the previous report, and conclude that further studies are necessary.

Adult↗
PubMed1991

Recovery of renal function in undifferentiated connective tissue disease after treatment with angiotensin-converting enzyme inhibitors.

Angiotensin-converting enzyme (ACE) inhibitor therapy has been reported to improve patient survival and promote recovery of renal function in the renal crisis of systemic sclerosis. In addition, an ACE inhibitor and a calcium channel blocker have been reported to control hypertension and reverse dialysis-dependent renal failure in a patient with undifferentiated connective tissue disease. We treated a patient with undifferentiated connective tissue disease who developed hypertension, pulmonary compromise, and renal failure requiring prolonged dialysis therapy. Due to allergy, the patient's hypertension could not be treated with ACE inhibitors initially, yet pulmonary function improved and renal function partially recovered with tenormin and minipress. When blood pressure became refractory to tenormin and minipress after 14 months of peritoneal dialysis, the patient was treated with lisinopril alone. Pulmonary function has remained stable and the patient has been off renal replacement therapy for 26 months, with a further substantial increase in creatinine clearance following treatment with lisinopril. The delayed and sustained recovery of renal and pulmonary function in the present case suggests undifferentiated connective tissue disease, like systemic sclerosis, may benefit from therapy with ACE inhibitors.

Acute Kidney Injury↗
PubMed1991

A reexamination of the relationship between myositis and malignancy.

The association between myositis and malignancy has long been appreciated. However, several recent reviews question the validity of this connection and the utility of pursuing an extensive search for occult malignancy in myositis. We report 9 patients fulfilling diagnostic criteria for myositis seen in the past 12 years. In each, a neoplasm was uncovered only in the course of a systematic search or, when initial investigation was deferred, at a later time. Of the 9 patients, 6 had dermatomyositis, 3 polymyositis. In 3 cases, solid tumors were diagnosed concomitantly with the myositis. In the remaining 6 patients, an advanced neoplasm was detected in the course of followup. In retrospect, some of these tumors might have been detected at an earlier and perhaps more treatable stage had a more rigorous initial malignancy search been pursued. Based on this experience, it is imperative that the clinician undertake a more exhaustive search for occult malignancy in patients with myositis than is currently recommended.

Adult↗
PubMed1991

The coexistence of active classic rheumatoid arthritis and AIDS.

The lack of previous reports documenting the coexistence of active rheumatoid arthritis (RA) and acquired immunodeficiency syndrome (AIDS) despite an abundance of other HIV associated autoimmune phenomena has generated a hypothesis regarding the necessity of the T4 cell for the development or continued activity of RA. We are the first to describe a patient in whom AIDS did not induce a remission of active synovitis. This observation emphasizes the complexity of the pathogenesis of RA.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Treatment of Wegener's granulomatosis with sulfamethoxazole-trimethoprim.

Sulfamethoxazole-trimethoprim may be an alternative or adjunctive treatment for Wegener's granulomatosis, as suggested by our experience with six patients. Two had limited Wegener's granulomatosis; one of these achieved remission with sulfamethoxazole-trimethoprim alone, and the second achieved remission with sulfamethoxazole-trimethoprim and prednisone. Four patients presented with sinus, pulmonary, and renal involvement. One patient initially treated with sulfamethoxazole-trimethoprim developed worsening renal function requiring cytotoxic therapy. Two patients initially treated with cytotoxic agents achieved remission coincident with the addition of sulfamethoxazole-trimethoprim for persistent sinus symptoms. One patient had relapse of pulmonary symptoms after achieving and maintaining a remission during treatment with sulfamethoxazole-trimethoprim alone. This experience suggests that sulfamethoxazole-trimethoprim may be an effective treatment for some patients with Wegener's granulomatosis and may be the only agent required. Patients require careful follow-up and still may need cytotoxic therapy.

Adult↗
PubMed1991

Clinical analysis of isolated angiitis of the central nervous system. A report of 11 cases.

Isolated angiitis of the central nervous system may occur more frequently, and with a more favorable outcome, than has previously been described. During the past 10 years, we diagnosed this condition in 11 patients ranging in age from 23 to 38 years whose initial presenting symptoms included severe headache, transient focal neurologic deficits, or seizures. The diagnosis was established in all cases by characteristic angiographic findings. All patients were initially treated with high-dose steroids; one patient later required the addition of an immunosuppressive agent. All patients responded initially to the steroids, and 10 were in clinical remission while receiving either a small dose of steroids or no medication after a mean follow-up of 33 months. One patient died 9 months after diagnosis, presumably of an intracerebral hemorrhage. This experience suggests that the diagnosis of isolated angiitis of the central nervous system should be considered in any young person complaining of persistent headache, transient focal neurologic deficits, or seizures. Angiography usually yields characteristic diagnostic findings. Treatment should consist of high-dose steroids, with the addition of immunosuppressive agents only in cases where steroids are ineffective.

Adult↗
PubMed1990

Hypothermia and systemic lupus erythematosus.

We describe an 18-year-old black woman with systemic lupus erythematosus (SLE) who presented with an exacerbation of polymyositis and neuropsychiatric symptoms. After starting prednisone she became hypothermic for 5 days. Hypothermia associated with SLE has been described in only 3 patients.

Adolescent↗
PubMed1990

Intraarticular noninflammatory free urate suspension (urate milk) in 3 patients with painful joints.

We describe 3 patients with painful intraarticular knee effusions composed of a viscous milky white suspension of monosodium urate crystals, in the absence of any cellular component. Two patients presented with acute bilateral knee pain. One patient presented with unilateral knee pain of gradual onset. All 3 patients had a history of ethanol abuse. Two patients had a history of gout. Two patients had chronic renal insufficiency, hypertension, and congestive heart failure. One patient had alcoholic cirrhosis. Two patients' pain responded to colchicine. One patient's discomfort was relieved only by repeated arthrocentesis. We conclude that intraarticular free urate can cause painful joints in the absence of an apparent inflammatory response.

Aged↗
PubMed1977

Binding of synthetic double-stranded DNA by serum from patients with systemic lupus erythematosus: correlation with renal histology.

Detection of antibody to double-stranded DNA by direct binding assays has proved useful in clinical management of patients with systemic lupus erythematosus (SLE). Recent confusion regarding specificity of these antibodies for SLE appears to be due, at least in part, to contamination of natural DNA preparations with nondouble-stranded DNA antigens. Measurement of binding of a synthetic, self-complementary DNA copolymer (dAT) rather than of natural DNA (KB) has been shown to obviate some of these difficulties, apparently because of freedom of dAT from nondouble-stranded DNA antigens. Among the advantages found in this way was a higher degree of specificity of antibodies to double-stranded DNA for clinically-judged active lupus nephritis than had been suspected. Since activity of nephritis is difficult to assess clinically, histologic data were sought to confirm these observations. Thirty-two kidney specimens were examined by light and/or electron microscopy. The degree of histologic activity and the amount and location of glomerular electron-dense deposits were semiquantitated blindly. The binding of both dAT and KB DNA was measured by the ammonium sulfate method. Correlation with the amount of electron-defense deposits was highly significant for dAT binding and somewhat less so for KB DNA binding as determined by both parametric and nonparametric statistical methods. Significant correlation with histologic activity was found for dAT but not KB DNA binding. These results are consistent with previous data and suggest that dAT binding may provide a useful, noninvasive means of clinically assessing both nephritis activity and the intensity of glomerular immune-complex deposition as reflected by the amount of electron-dense deposits. If it can be confirmed that the latter provides long-term prognostic information, then dAT binding (and perhaps its reponse to therapy) may also prove of value in this regard.

Adenosine Monophosphate↗
PubMed1977

Interstitial pneumonitis complicating rheumatoid arthritis. Sustained remission with azathioprine therapy.

A patient with classic rheumatoid arthritis developed biopsy-proven diffuse interstitial pulmonary fibrosis and ventilatory insufficiency which appeared to be irreversible. The administration of azathioprine coincided with significant immediate improvement in pulmonary function and clinical status. During five years of continuous azathioprine therapy, progressive improvement in lung function has been accompanied by marked deterioration of the rheumatoid joint disease, suggesting that the pulmonary and joint lesions of rheumatoid disease may not be mediated by the same pathways.

Arthritis, Rheumatoid↗
PubMed1976

Detection of anti-DNA antibody using synthetic antigens. Characterization and clinical significance of binding of poly (deoxyadenylate-deoxythymidylate) by serum.

Virtually all preparations of DNA used to detect antibody to native DNA (nDNA) by binding assays have been found to be subtly contaminated by single stranded DNA. Because recent DNA binding data have directly challenged the unique role previously attributed to these antibodies in systemic lupus erythematosus (SLE), resolution of the consequent ambiguity is of theoretical and practical importance. It is proposed that a synthetic nDNA molecule (dAT) might circumvent this difficulty by being antigenically equivalent to nDNA while, on theoretical grounds, lacking significant contamination with single stranded DNA or other cellular antigens. These expectations were generally confirmed by biochemical and immunological analyses. In clinical studies, sera from 124 pateints with SLE and from controls were examined for their ability to bind dAT. In contrast to results with KB binding, patients with non-SLE rheumatologic disorders were indistinguishable from normals by dAT binding. dAT binding was elevated in 85% of sera from SLE patients with clinically-judged active nephritis but in only 9% of those with inactive renal disease. Active non-renal disease, including cerebritis, was not associated with increased dAT binding. Individual non-lupus sera which bound increased amounts of KB DNA, failed to bind dAT. It is suggested that such binding resulted from contaminating non-nDNA antigens. When elevated, dAT binding, like KB binding, varied with disease activity and might thus be useful as a parameter thereof. In several patients elevated dAT binding led to the finding, on biopsy, of clinically silent, active, diffuse proliferative nephritis. It is concluded that use of synthetic nDNA antigens such as dAT may offer theoretical and practical advantages over naturally-derived preparations in detecting anti-nDNA, both clinically and for investigational purposes. Also, caution is urged in interpreting DNA binding data derived from incompletely characterized systems, particularly with regard to the occurrence of anti-nDNA antibodies in serum.

Adult↗
PubMed1976

Multifocal fibrosis involving the thyroid, face, and orbits.

Our patient had fibrous thyroiditis with unusual facial and retroorbital fibrosis. Systemic fibrosis, which is more typical of the multifocal fibrosclerosing syndromes, was not evident. Treatment with steroid alone, surgery, and radiotherapy failed to maintain a remission. The combination of steroid and cyclophosphamide therapy appears to have been successful.

Bone Diseases↗