Primary synovial chondromatosis: an elemental investigation of a rare skeletal pathology.

BACKGROUND: Primary synovial chondromatosis (PSC) is a rare idiopathic pathology characterised by the formation of osseocartilaginous nodules within synovial joints, tendons, or bursae. The mineralisation pattern of PSC nodules is poorly understood and has yet to be investigated using elemental analysis. Mapping this pattern could elucidate the progression of the disease. MATERIALS AND METHODS: Primary synovial chondromatosis nodules discovered during dissection of a formalin fixed donor were analysed. Scanning electron microscopy paired with energy dispersive X-ray spectroscopy (SEM-EDS) was used to quantify calcium and phosphorus levels to distinguish mineralised components from cartilage, indicated by increased carbon and oxygen concentrations. RESULTS: Nine nodules with average dimensions 1.76 cm × 1.25 cm were identified in the semimembranosus bursa. SEM-EDS demonstrated increased calcium phosphate levels in nodular cores, while outer margins contained primarily carbon and oxygen. Quantification of these elements revealed nodular peripheries to contain 68.0% carbon, 30.2% oxygen, 0.8% calcium, and 1.0% phosphate, while cores were comprised of 38.1% carbon, 42.1% oxygen, 14.1% calcium, and 5.7% phosphate. CONCLUSIONS: Nodules were found to have mineralised cores embedded within a cartilaginous matrix. This pattern suggests disease progression is facilitated by endochondral ossification, opening the potential for new therapeutic techniques.

[Use of Cyclosporine A and new immunosuppressive drugs in the therapy of glomerulonephritis]. / Zastosowanie Cyklosporyny A oraz nowych leków immunosupresyjnych w leczeniu pierwotnych glomerulopatii.

Multiple clinical trials have been undertaken during last years to assess indications, efficiency and safety of glomerulonephritis treatment with new immunosuppressive drugs as cyclosporine (CsA, Mycophenolate Mophetil (MMF) and Tacrolimus (FK 506). The main indication for cyclosporine is nephrotic syndrome resistance to steroids and cytotoxic agents, steroid-dependent and multi-relapsing cases with serious toxic side effects or with contraindications for steroids and cytotoxic drugs. CsA was administered at the dose of 4-5 mg/kg/day in adults and 5-6 mg/kg/day in children. The best results were achieved with minimal change disease. The durable remission occurred in 70-80% of cases of steroid-sensitive nephrotic syndrome and in 20-30% of steroid-resistant forms. There was a lower rate of remission and a high risk of cyclosporine nephrotoxicity in other types of glomerulonephritis. Therefore CsA, MMF and FK506 remain a late therapeutic option for patients with these types of glomerulonephritis and severe clinical course. As the long-term CsA therapy may be complicated by acceleration of renal fibrosis, a renal biopsy is mandatory before its administration.