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Biomedical subjects

H Spiera

Publications and source records attributed to H Spiera.

At least 37 records · Page 2Linked to original sources
PubMed1995

Regression of the nephrotic syndrome in rheumatoid arthritis and amyloidosis treated with azathioprine. A case report.

Secondary or reactive (AA) amyloidosis is a well-known complication of certain rheumatic diseases, particularly rheumatoid arthritis (RA). This case report describes a patient with RA complicated by amyloidosis and the nephrotic syndrome, which regressed after treatment with azathioprine. The AA amyloidosis was documented by renal and lymph node biopsies and by the presence of proteinuria. Evaluation for other etiologies of proteinuria was negative. After treatment with azathioprine, the proteinuria resolved and the serum albumin level increased from 1.9 mg/dl to normal. This is the first published report of azathioprine therapy resulting in a reversal of the nephrotic syndrome in a patient with RA and secondary amyloidosis.

Amyloidosis↗
PubMed1995

Eosinophilic temporal and systemic arteritis.

We describe a 39-year-old patient with an unusual type of bilateral temporal arteritis characterized histologically by inflammation, diffuse eosinophilic infiltration, destruction of elastic tissue, and fibrosis. In addition, the patient had a history of systemic vasculitis, peripheral eosinophilia, eosinophilic lymphadenitis, and membranous glomerulonephritis. The patient has been followed up for 14 years and is well controlled on moderate doses of steroids. We propose that this patient suffers from an immune reaction to an unknown, possibly infectious, antigen.

Adult↗
PubMed1995

Panniculitis of pancreatic disease masquerading as systemic lupus erythematosus panniculitis.

We describe a patient with systemic lupus erythematosus (SLE) who presented with severe refractory pannicular lesions diffusely involving the buttocks and lower extremities. Due to the severity of these lesions, a biopsy was performed, which implicated panniculitis associated with pancreatic disease, rather than lupus panniculitis. Serum amylase was normal, but the serum lipase was markedly elevated. An abdominal computerized tomographic scan demonstrated a pancreatic mass, which upon laparotomy was found to be an acinar cell carcinoma. After resection of the mass, her symptoms improved, where they had not responded to prior immunosuppressive therapy. She has subsequently remained well without recurrence of the disease. This case illustrates that an uncharacteristic presentation of panniculitis in a patient with lupus does not necessarily imply lupus panniculitis, and a biopsy is imperative to distinguish other sometimes life threatening etiologies.

Carcinoma, Acinar Cell↗
PubMed1995

Position 26 of the first domain of the HLA-DQB1 allele in post-silicone implant scleroderma.

OBJECTIVE: To determine whether women with scleroderma (systemic sclerosis, SSc) and silicone implants have the same or distinctive immunogenetic findings compared to those reported for idiopathic scleroderma. METHODS: In this case-control study, 9 Caucasian women with SSc and silicone implants (7 breast, 1 chin, 1 toe) and 128 healthy Caucasian controls were typed for HLA class II (DRB1,3,4,5, and DQB1) by DNA polymerase chain reaction (PCR) sequence specific oligonucleotide probes (SSOP). RESULTS: All women with SSc had HLA-DQ5 or DQ7 (DQB1*0301). These 2 alleles have glycine (Gly) or tyrosine (Tyr), and not hydrophobic leucine (Leu), at position 26 in the 2nd hypervariable region of the DQB1 first domain. The increased frequency of at least one Leu 26 negative allele (Gly + or Tyr +) in the women with SSc (100%) compared with controls (73%) was not statistically significant. In contrast, the low frequency of one Leu 26+ allele in the patients (28 vs 57%, p = 0.03, RR = -3.3) and 2 Leu 26+ alleles (0 vs 35%, p = 0.03, RR = -10.4) was significant. CONCLUSION: The presence of Gly 26 or Tyr 26 in the HLA-DQB1 first domain in our cases with SSc and silicone implants is consistent with immunogenetic findings reported in Caucasian with idiopathic SSc anticentromere autoantibodies. Whether all the immunogenetic features in SSc associated with silicone implants remain indistinguishable from those seen in idiopathic.

Alleles↗
PubMed1994

Correlation of liver and skin histopathology with serology in a patient with cutaneous vasculitis and hepatitis C infection.

A strong body of evidence supports a viral etiology for many forms of vasculitis. Recent literature supports the association of the hepatitis C virus (HCV) with essential mixed cryoglobulinemia. We describe a case of cutaneous vasculitis associated with simultaneous active hepatic inflammation demonstrated by skin and liver biopsies. Immunofluorescence, hepatitis C serology, and study of the cryoprecipitate all confirmed HCV infection and deposition of immune complexes. Treatment with interferon alpha-2b was extremely effective as compared with prednisone and colchicine. This case provides further evidence that active hepatic infection with HCV may be a cause of cryoglobulinemia and cutaneous vasculitis. It also suggests that further studies of antiviral therapy for vasculitis should be explored.

Female↗
PubMed1994

ACTH revisited: effective treatment for acute crystal induced synovitis in patients with multiple medical problems.

OBJECTIVE: To determine the effectiveness of adrenocorticotropic hormone (ACTH) for acute gouty arthritis and pseudogout in a population of patients with multiple coexisting medical problems. METHODS: We retrospectively reviewed our experience with parenteral ACTH 40 or 80 units intravenous, intramuscular, or subcutaneous tid with tapering in the treatment of 38 patients. Thirty-three patients had documented acute gout and 5 patients had documented acute pseudogout. A total of 43 episodes of acute crystal induced synovitis were treated. The indications for using ACTH included congestive heart failure, chronic renal insufficiency, gastrointestinal bleeding, or no response to NSAID: RESULTS: All episodes of pseudogout resolved in an average of 4.2 days. Of the episodes of acute gout, 97% resolved in an average of 5.5 days. Although mild hypokalemia, hyperglycemia, fluid retention and rebound arthritis occurred as adverse effects, none was severe and all were easily controlled. CONCLUSION: ACTH is a safe and effective treatment for acute gout and pseudogout, especially in patients with multiple medical problems.

Adrenocorticotropic Hormone↗
PubMed1994

Silicone gel filled breast implants and connective tissue disease: an overview.

OBJECTIVE: To review the literature examining the association of silicone gel filled implants and connective tissue disease. METHODS: Computerized literature searches and manual review of bibliographies. RESULTS: Numerous concerns have arisen regarding the safety of silicone gel filled breast implants. The structure of these prostheses is reviewed. Silicones are not biologically inert. Injectable as well as implantable silicones have proven capable of eliciting inflammatory and fibroproliferative responses. Silicone leakage from silicone gel filled implants is well documented as is distant migration of silicone in the host. In the past decade, over 60 cases of connective tissue disease following mammoplasty with silicone gel filled implants have been reported. About half of these patients developed scleroderma or scleroderma-like illnesses. This reported overrepresentation of scleroderma compared to other rheumatic diseases mimics the Japanese experience with injectable silicones. Possible biological rationale for the association is presented. CONCLUSION: The physical and biological properties of silicone gel filled implants and their behavior in vivo is compatible with the hypothesis that they may contribute to the development of connective tissue disease. The association seems most likely with scleroderma; however, there is as yet inadequate epidemiological data to definitively establish causality.

Animals↗
PubMed1993

Autoantibodies directed against different classes of Fc gamma R are found in sera of autoimmune patients.

Serum samples from 147 patients with different systemic autoimmune diseases (SLE, Sjögren's syndrome, and progressive systemic sclerosis) were tested for anti-Fc gamma R activity using mouse rFc gamma RII in an ELISA. High reactivity compared to normal individuals was found for patients with all three diseases. The anti-Fc gamma R antibody was purified from several serum samples by affinity chromatography on a Sepharose column coupled with denatured, murine rFc gamma RII. Both IgM and IgG antibodies were found. To analyze the specificity of the affinity-purified autoantibody, cells (human neutrophils, IFN-gamma-stimulated neutrophils, monocytes, and the THP-1 monocytic cell line) that express different combinations of Fc gamma R (CD64, CD32, CD16) were stained with the affinity purified Ig. Ig directed against all three types of Fc gamma R were found. The results may reflect on the role of Fc gamma R-specific antibodies in the pathology of autoimmune diseases.

Animals↗
PubMed1993

Myocarditis as a complication in scleroderma patients with myositis.

This report describes six diffuse scleroderma (PSS) patients with skeletal muscle myositis accompanied by severe myocarditis, diagnosed by CPK-MB elevation in conjunction with severe left ventricular (LV) hypokinesis. Although the myositis improved with steroid therapy in all patients, those treated with steroids alone died due to progressive LV failure. This experience suggests that the LV dysfunction in PSS patients with myositis may have an inflammatory component. Since the myocarditis may not be clinically apparent initially, it is suggested that CPK-MB fractionation and studies of LV function are undertaken in all PSS patients with myositis. The optimal treatment of this disorder, however, has yet to be determined.

Adult↗
PubMed1993

Systemic inflammatory disorder related to fibrous breast capsules after silicone implant removal.

Silicone breast implants have been associated with connective-tissue inflammatory syndromes such as systemic sclerosis, and as with other artificial breast prostheses, fibrous capsules tend to form around the implants. The capsular tissue is generally considered inert and typically is left in situ when the prostheses are explanted. We report a patient who formed symptomatic bilateral submammary cysts associated with pain, swelling, arthralgia, fever, axillary lymphadenopathy, accelerated erythrocyte sedimentation rate, and antinuclear antibody following removal of intact silicone breast implants without capsulectomy. Clinical improvement followed removal of the capsules, which histologically displayed fragments of silicone, fibrous tissue, and inflammatory cells. Our experience suggests that when silicone breast implants are thought to be the cause of a clinical inflammatory syndrome characterized by mammary pain, swelling, arthralgia, or serologic abnormalities, consideration should be given to removing the capsules entirely so that the chance of a perpetuating reaction will be reduced.

Arthritis↗
PubMed1993

Autoantibodies to the protein core of vascular basement membrane heparan sulfate proteoglycan in systemic lupus erythematosus.

Vascular heparan sulfate proteoglycan (vHSPG) has an important role in the normal vasculature, including hemostasis, lipolysis and other vascular functions. These functions are mediated by both the glycosaminoglycan and protein core moieties of vHSPG. Autoimmunity to vHSPG has been demonstrated to play a role in vascular injury in animal models, and is present in patients with autoimmune vascular disease. However, most previous studies of human autoimmunity to vHSPG have only investigated heparan sulfate glycosaminoglycan epitopes. In the current investigations, autoantibodies to the protein core of vHSPG in sera from patients with systemic lupus erythematosus (SLE) were investigated. vHSPG protein core was prepared by chemical deglycosylation. Competitive immunoinhibition ELISA and immunoblotting immunoassays were established employing monoclonal antibodies to vHSPG protein core. SLE sera were demonstrated to contain IgG autoantibodies reactive with the vHSPG protein core by immunoblotting. Human autoantibodies to vHSPG protein core were not inhibited by heparan sulfate confirming their protein core reactivity. Competitive immunoinhibition studies employing a solid phase radioimmunoassay also confirmed the reactivity of human sera with vHPSG protein core. By ELISA, a significant increase in the occurrence of anti-vHSPG protein core antibodies was noted in SLE sera. While most previous investigations have demonstrated autoimmunity to heparan sulfate, the presence of IgG autoantibodies to vHSPG protein core demonstrates that the entire vHSPG proteoglycan is the target of autoimmunity in SLE.

Animals↗
PubMed1993

Scleroderma following silicone implantation: a cumulative experience of 11 cases.

In the 3 years since the initial report of 5 patients who developed scleroderma in association with silicone breast implants, 6 additional silicone implant recipients have presented with scleroderma. Two of these patients received silicone chin implants. A total of 5 patients elected to remove their prostheses. Two patients have had marked improvement of cutaneous scleroderma. The other 3 patients, however, died secondary to progressive visceral involvement. This cumulative experience of 11 cases of scleroderma following silicone implantation suggests additional evidence of an association between the 2. Careful case control studies over an extended period of time are required to determine the incidence of this outcome.

Adult↗
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↗