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Biomedical subjects

D D'Cruz

Publications and source records attributed to D D'Cruz.

71 records · Page 4Linked to original sources
PubMed1992

Low dose methotrexate treatment in adult Still's disease.

Six patients with adult Still's disease who had either failed to respond to or had adverse effects from previous therapy were given weekly low dose methotrexate (MTX) therapy. They were followed for 4-28 months (mean 14 +/- 9 months). At 12 months of therapy 3 patients were evaluated as having a complete response. One patient had a partial response to MTX after 7 months. Therapy was discontinued in 1 patient at 4 months due to flares of rash and arthralgias after each MTX administration. One patient failed to respond to therapy despite maximum dosage, but was able to reduce his corticosteroid dose. MTX may be a useful therapy to consider in patients with adult Still's disease who are resistant to conventional therapy and may allow a reduction in the concomitant dose of corticosteroids.

Adult↗
PubMed1991

Urticarial vasculitis in a connective tissue disease clinic: patterns, presentations, and treatment.

Findings in 27 patients with typical skin lesions of urticarial vasculitis (UV) who were seen at a connective tissue disease clinic over a 5-year period (1986 to 1990) are reviewed. The majority suffered from systemic lupus erythematosus (SLE) or from "lupus-like" disease (18 patients), 1 from "mixed" connective tissue disease (MCTD), and 5 from primary UV. All of the latter patients had normal serum complement levels (normocomplementemic urticarial vasculitic syndrome; NUVS). No patients with hypocomplementemic UV were encountered. Two patients suffered from necrotizing vasculitis (polyarteritis nodosa, Wegener's granulomatosis); one had a C1-esterase inhibitor deficiency and also demonstrated an immunoglobulin G paraproteinemia. Angioedema occurred in many patients and could not be used as a differential diagnostic feature. The course of the illness was chronic in most patients, lasting for up to 23 years, and the response to therapy was unpredictable, erratic, and unsustained. The use of intravenous "pulse" methylprednisolone, cyclophosphamide, or high-dose oral steroids helped selected patients. Colchicine was dramatically effective in one patient with NUVS of 15 years duration. Azathioprine was not beneficial. None of the five patients with NUVS suffered from severe systemic involvement or renal disease, confirming observations by others that this form of UV represents a milder example of the condition.

Adult↗
PubMed1991

Polymyositis complicating D-penicillamine treatment.

Although there is good evidence that D-penicillamine can induce polymyositis, the exact pathogenic mechanism remains unclear. We report two patients with psoriatic arthritis and primary biliary cirrhosis respectively, who developed polymyositis while receiving D-penicillamine treatment for their primary diseases. Whether D-penicillamine treatment was the sole cause of polymyositis or acted as a trigger for the development of a secondary autoimmune disease is discussed.

Adult↗
PubMed1991

Antiendothelial cell antibodies in patients with the antiphospholipid syndrome.

Antiendothelial cell antibodies (AECA) have been detected in 20 out of 30 patients (67%) with thrombosis associated with antiphospholipid antibodies. Seven patients had systemic lupus erythematosus and 13 had the "primary" antiphospholipid syndrome. Seven patients had both IgG and IgM AECA, 9 had IgG AECA only, and 4 had only IgM AECA. None of 30 control patients with thrombotic events not related to antiphospholipid antibodies had a positive titre of AECA (P less than 0.001). No correlation between AECA, antinuclear antibodies, anti-dsDNA antibodies, anti-neutrophil cytoplasm antibodies, precipitating antibodies to soluble nuclear and cytoplasmic antigens or complement components was found. The possible role of these AECA in the pathogenesis of thrombotic events is discussed.

Adult↗
PubMed1990

[Wegener's granulomatosis. New aspects of diagnosis, treatment and prognosis].

In its classical form, Wegener's granulomatosis (WG) is characterized by acute granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis, and when untreated, mortality is high. The use of cyclophosphamide and prednisolon has improved the prognosis dramatically, while azathioprine and trimetoprim-sulfamethoxazole are of limited therapeutic value in these patients. Only 1/3 of subacute and chronic cases show renal impairment, and the prognosis is better in limited Wegener's granulomatosis without renal disease than in the classical form. This case-report and review takes up various new aspects of diagnosis, treatment and prognosis.

Adult↗
PubMed1990

Adult-onset polymyositis-dermatomyositis: description of 25 patients with emphasis on treatment.

A retrospective study of 25 patients with polymyositis-dermatomyositis (PM-DM) is analyzed with special attention to the effects of therapy and follow-up. All patients (100%) complained of muscle weakness and 68% of these demonstrated typical skin changes of DM. All patients, except 2, received corticosteroids at the onset of the disease, 23 were treated with azathioprine, 7 received cyclophosphamide, 4 methotrexate, and 1 had total body irradiation. Among the patients adequately treated with azathioprine, 75% had a good response, but 5 patients did not improve. Cyclophosphamide was used subsequently in 2, with a satisfactory response in 1. Another patient had a striking response to oral methotrexate, and total body irradiation helped to improve another patient. Although high dose corticosteroids were the preferred starting medication for the treatment of PM-DM, it is important to detect those patients who do not respond adequately and/or develop side effects. In these circumstances, the prompt use of immunosuppressive agents appears justified.

Adult↗
PubMed1986

Heterozygous C2 deficiency associated with angioedema, myasthenia gravis, and systemic lupus erythematosus.

We describe a patient with myasthenia gravis, systemic lupus erythematosus, and angioedema associated with heterozygous complement factor 2 (C2) deficiency. The significance of this association is controversial, though the association of C2 deficiency with certain histocompatibility antigens suggests possible linkage to immune response genes. To our knowledge this is the first report of heterozygous C2 deficiency in association with this combination of 'autoimmune' disorders, and we discuss the aetiological implications.

Adult↗
PubMedDate not supplied

Anterior ischaemic optic neuropathy in Wegener's granulomatosis.

Is reported a case of Wegener's granulomatosis originally presenting with a clinical picture of temporal arteritis. The presentation included an arteritic anterior ischaemic optic neuropathy occurring with no demonstrable orbital inflammation. The treatment of these two systemic vasculitides is quite different, however, and this case emphasizes the importance of questioning the original diagnosis if the clinical course of the disease does not progress as anticipated.

Female↗
PubMedDate not supplied

Neurological manifestations of anti-phospholipid (Hughes) syndrome.

In the 21 years since the description of the antiphospholipid syndrome (APS), neurological features have become recognised as some of the most important and common aspects of the syndrome. Recognition of the relationship between APS and central nervous system impairment could open the door to improved treatment and outcomes in some neurological syndromes.

Antiphospholipid Syndrome↗
PubMedDate not supplied

Lupus nephritis: the significance of serological tests at the time of biopsy.

We reviewed the initial serological data of 50 patients with biopsy-proven lupus nephritis. As compared with a group of lupus patients without nephritis, patients with nephritis had lower serum complement C3 (p less than 0.05) and C4 (p less than 0.005) levels and higher serum DNA binding activity (p less than 0.001). The frequency of rheumatoid factor, antiphospholipid, anti-ENA, and fluorescent antinuclear antibodies was similar in both groups. We correlated the serological data of the patients with nephritis with the clinical severity of their disease. Using a functional staging system based on the serum albumin and creatinine levels at the time of biopsy, we found that patients with functionally milder disease (proteinuria without nephrotic syndrome or renal failure) had higher C3 (p less than 0.05) and lower DNA binding (p less than 0.005) than patients in the more severe functional classes (nephrotic syndrome with or without renal failure). In contrast, C4 levels were always very low, irrespective of functional severity. We also correlated the serological data with the pathological findings. Patients suffering from diffuse proliferative nephritis had higher DNA binding values than patients with focal proliferative (p less than 0.01) or membranous (p less than 0.001) nephritis. By contrast, complement levels were not correlated with the severity of biopsy changes. Taken together, the data presented here suggest that C3 and DNA binding, but not C4, correlate with the clinical severity of lupus nephritis at presentation whereas DNA binding, but not complement levels, correlates with the severity of pathological changes.

Adult↗
PubMedDate not supplied

Immunosuppressive therapy in lupus nephritis.

OBJECTIVE: To evaluate the outcomes and side effects of immunosuppressive therapy in patients with lupus nephritis. PATIENTS AND METHODS: Thirty-nine patients with lupus nephritis assessed between 1988 and 1993 with a median follow-up of 46 months (range 12-60 months) were studied. Lupus nephritis was biopsy-proven in 37 patients. Patients received a median of 3 (500 mg) weekly pulses of intravenous cyclophosphamide followed either by azathioprine (n = 32) or oral cyclophosphamide (n = 7). All patients received oral prednisolone. The time from biopsy to renal insufficiency, defined by doubled serum creatinine and/or end stage renal failure, was used to assess outcome. RESULTS: There were significant improvements in the median changes of all major laboratory parameters. Serum creatinine levels did not change significantly. The prednisolone dose was significantly reduced during the follow-up period. OUTCOME: renal function remained stable in 26 (67%) and deteriorated despite therapy in 13 (33%) patients. 6/13 (42%) of these patients had impaired renal function at the time of biopsy. The adverse effects of intravenous cyclophosphamide seen were Herpes zoster (1), transient leucopenia (2), rash (1) and fatal septicaemia (1); of azathioprine urinary infections (3), leucopenia (5), rash (1) and increased liver enzymes (1); and of oral cyclophosphamide ovarian failure (4), Herpes zoster (3), haemorrhagic cystitis (1), and fatal septicaemia (1). CONCLUSIONS: Therapy with weekly low dose intravenous pulse cyclophosphamide to induce remission, followed by azathioprine appears to be useful in preserving renal function in patients with diffuse proliferative lupus nephritis. In comparison to other studies, the reduced incidence of ovarian failure using this regimen was striking.

Administration, Oral↗
PubMedDate not supplied

Markers of vascular damage in lupus pregnancy.

OBJECTIVE: To study serum levels of von Willebrand factor antigen (vWF:Ag) and anti-endothelial cell antibodies (AECA) in pregnancies with systemic lupus erythematosus (SLE) compared to healthy pregnancies and to evaluate their correlation with disease activity and fetal outcome. METHODS: Twenty-four patients with SLE were compared with 26 healthy pregnancies. vWF:Ag and AECA levels were measured by ELISA. RESULTS: In lupus pregnancies vWF:Ag and AECA levels were significantly elevated compared to healthy pregnancies (vWF:Ag: 255 +/- 160%, 10/24 positive vs 135 +/- 59%, 1/26 positive, p = 0.006; AECA Binding Index: 36 +/- 29%, 5/24 positive vs 17 +/- 16%, 1/26 positive, p = 0.002). When samples taken during follow-up were evaluated, vWF:Ag was positive in 21/27 (78%) of the samples during exacerbations compared to 4/25 (16%) in remission. Only 5/27 (19%) of the samples during exacerbations and none during remission were positive for AECA. Samples during three pregnancy failures were positive for vWF:Ag, but not for AECA. Of the 13 samples taken from the mothers of 4 babies with neo-natal lupus, 10 were positive for vWF:Ag and three for AECA. No correlations of anti-cardiolipin antibodies (aCL) were seen either with vWF:Ag or AECA titres. CONCLUSION: Compared to healthy pregnancies, vWF:Ag and AECA levels are increased in lupus pregnancies. vWF:Ag levels also correlated with active disease.

Abortion, Spontaneous↗