STEROID-PULSE THERAPY IN PEMPHIGUS VULGARIS LONG TERM EOLLOW-UP

Background. Patients with pemphigus vulgaris may occasionally present the highly active, widespread form of the disease, and/or maybe resistant to conventional oral steroid therapy, and/or manifest significant side effects from the prolonged use of high oral prednisone dosage. Our purpose was to evaluate the efficacy of steroid “pulse” therapy (PT) in these patients. Methods. Eight patients were given alternate-day, one hour, infusions of 8, 9, or 10 mg/kg methylprednisolone Na succinate. Oral prednisone and a second immunosuppressive agent were simultaneously administered; these were rapidly decreased when control of the disease was achieved. Results. All patients initially responded well to therapy. The disease recurred in four patients after 3, 4, 9, and 16 months of remission, respectively. Three of these patients were treated again with pt and went into remission. None of the patients who received cyclophosphamide had a recurrence. One patient died of cardiac arrest, 12 days after pt. On a follow-up of 59 ± 25 months since the last relapse, patients continue in remission. Conclusions. Pulse therapy is recommended as an adjunct to the total management plan of severely affected patients with pemphigus vulgaris. The addition of cyclophosphamide may prevent the disease from recurring. Alternate-day small-quantity bolus infusions over 16–20 days, may be equally effective as the administration of 15 mg/kg/daily for 3–5 days. The risk of cardiac arrest exists even with this, less aggressive form of pt therapy. A medical history of supraventricular arrhythmias may be considered a risk factor.     

Investigation of childhood central nervous system vasculitis: magnetic resonance angiography versus catheter cerebral angiography

Aim We compared the clinical utility of magnetic resonance angiography (MRA) to catheter cerebral angiography (CA) in the investigation of children with suspected central nervous system (CNS) vasculitis. Method Single‐centre retrospective review of children with a suspected diagnosis of CNS vasculitis studied with both MRA and CA. MRA and CA‐detected abnormalities (location, multiplicity, and morphology) were compared; sensitivity and specificity were calculated on a per lesion and per patient basis for MRA, with CA as the reference standard. Results Findings in fourteen patients (median age at presentation of 5y 10mo [range 1y 5mo–14y 5mo]; eight males, six females) relating to sixteen paired studies of MRA and CA were reviewed. CA‐detected lesions were commonly bilateral (13/16 studies, p<0.05), and likely to be proximally distributed (15/16 studies, p<0.05).The sensitivity and specificity of MRA for CA lesion detection was 63% (95% confidence interval [CI] 48–78) and 89% (95% CI 81–93), respectively with moderate agreement between the two modalities (κ=0.51, 95% CI 0.37–0.66). The majority of the false negative observations involved the posterior circulation (9/14). The overall sensitivity for MRA diagnosis of vasculitis per patient was 94% (95% CI 67–99). Interpretation MRA failed to identify all lesions detected on CA, particularly those in the posterior circulation. MRA is a reasonable initial modality in the investigation of suspected CNS vasculitis but in cases of abnormal parenchymal MRI and normal MRA, CA should be considered.     

SERUM CYFRA 21–1 IN PATIENTS WITH INVASIVE BLADDER CANCER AND ITS RELEVANCE AS A TUMOR MARKER DURING CHEMOTHERAPY

Purpose:

Previous studies have shown that serum levels of the degradation products of cytokeratins could be used as surrogate markers in the diagnosis and followup of patients with solid tumors, including tumors of the bladder.

Materials and Methods:

The soluble cytokeratin 19 fragment CYFRA 21-1 was measured by solid phase radioimmunoassay in the serum of 142 patients with invasive transitional cell cancer of the bladder. Of the patients 56 had clinical stage I to III locally confined disease (T1-4aN0M0) and 86 had stage IV metastatic disease with lymph node and/or distant metastases. A control group consisted of 33 healthy volunteers. In a subgroup of 49 patients with metastatic disease receiving combined platinum based chemotherapy serum CYFRA 21-1 was determined prior to the initiation of therapy and after the documentation of response.

Results:

Abnormal CYFRA 21-1 was observed in 7% of patients with locally invasive disease and in 66% of those with metastatic disease (p <0.0001). There was no correlation of CYFRA 21-1 with tumor differentiation. Patients with abnormal CYFRA 21-1 showed statistically significant worse median overall survival. Moreover, in the subgroup of patients with metastatic disease receiving chemotherapy CYFRA 21-1 levels correlated with the response to treatment.

Conclusions:

Patients with transitional cell cancer of the bladder with evidence of distant metastases showed a significant increase in serum CYFRA 21-1. During chemotherapy CYFRA 21-1 appears to be a potentially sensitive and useful indicator for monitoring treatment response.     

TREATMENT OF ORAL PEMPHIGUS VULGARIS

Background. Oral pemphigus is considered to represent either an initial stage of pemphigus vulgaris that should be treated with high doses of immunosuppressive drugs to prevent its spread to the skin or a variety of the disease that does not need aggressive treatment. The absence of a widely accepted therapeutic method and the obscure nature of this disease prompted this study. Methods. Twenty-eight patients with oral pemphigus were randomly divided into three groups. The first group received only steroids at a dose of 40 mg of prednisone equivalent. In the other two groups the same dose of steroids was administered, but combined with either 100 mg of cyclophosphamide or 5 mg/kg of cyclosporine as an adjuvant. Direct immunofluorescence studies were performed on specimens obtained from both buccal mucosa and oral skin. Results. There was no significant difference in the duration of treatment required to achieve remission and in the relapse rate among the three groups. The incidence of complications was higher with combination treatment. Deposited immunoreactants were detected with equal frequency in specimens obtained from the buccal mucosa and normal skin. Conclusions. Oral pemphigus most likely represents an initial stage of a disease that can become generalized. Administration of moderate doses of steroids alone is effective in controlling the disease.