The frequency of osteoporosis in patients with pemphigus vulgaris

Currently, suppressing synthesis of autoantibodies for remission of disease in a short period is the first step in management of patients with pemphigus. For this reason, therapy is based on non-specific immunosuppression. The first choice of treatment in pemphigus vulgaris is systemic glucocorticoids if there is no absolute contraindication. Prior to the use of glucocorticoids in 1950?s, the natural history of pemphigus vulgaris was a devastating disease, with a 50% mortality at 2 years, and almost 100% at 5 years. Today, mortality rate is less than 5%, but morbidity rate has been increasing due to long-term immunosuppression therapy.Unfortunately, the high doses and prolonged administration of corticosteroids which often needed to control the disease result in numerous side effects, many of which are serious. Osteoporosis is the one of the major and avoidable adverse effect of long term use of steroid treatment. Patients, who are planned to receive long term steroid treatment, should be regularly examined for bone loss.In this study we aim to investigate osteoporosis and compare its rate between patients with pemphigus vulgaris using systemic corticosteroids and normal population.We enrolled patients with pemhigus vulgaris (n=40) and matched controls (n=34) who were admitted to Dermatology Clinic of Dicle University Hospital in the period of January 2008- March 2009. Both patient and control group cases were investigated for the history of fracture. Bone mineral density measurements were obtained by DEXA for both groups. Bone turnover markers in 24-hour urine with blood serum bone parameters, and biochemical hormonal measurements were examined to exclude the secondary osteoporosis. Patients in both groups who have secondary osteoporosis were excluded.Mean ages were 44.88 ± 11.68 years and 48.97 ± 15.06 years in patient and control groups respectively. There was no significant difference in terms of age, sex, body mass index, waist-hip ratio, menopause rate, alcohol, drinking and smoking between two groups. Mean follow-up period was 52 months, changing between 2- 168 months. Patients were categorized into three groups according to steroid dosage. Thirty five percent of patients was treated with low dose (<80mg/day), 52,5% with moderate dose (80-120mg/day), and 12,5% of patients with high dose (>120mg/day). The mean exposure of steroid was 30 months. The results of femur BMD values were not differ between two groups while there is a significant difference in lumbar T (p=0.034) and Z scores (p=0.006). Osteoporosis, osteopenia and normal DEXA rate were %32.5, %32.5 and %35 respectively. Corresponding rates in the control group were found to be %18, %23 and %59 respectively. Rate of fracture in patients group was more than control group, and this was statistically significant (p=0.004). The acquired inactive life style was statistically different in patient group compared with control group (p=0.007). The bone resorption markers like urine calcium, pyridoline and deoxypyridoline levels were found to be high in patient group (p=0.003, p=0.018, p=0.006 respectively). Urine hydroxypyridoline levels were not different between two groups (p=0.287).In patient group, we didn?t find any significant relation between steroid dose, treatment time, osteoporosis, osteopenia, and bone resorption markers.Patients with pemphigus vulgaris receiving systemic corticosteroids should be initially examined by DEXA. Patients who have normal DEXA results should be given calcium and vitamin D prophylactically and suggestions should be made for preventing osteoporosis. Patients on systemic steroid treatment should also be suggested yearly DEXA examination to evaluate BMD while on treatment. Follow up and therapy of patients with low BMD values are mandatory. Anti-resorptive drugs if needed should be instituted with consultation of Endocrinology clinic.