ABSTRACT
INTRODUCTION: Collagenofibrotic glomerulopathy or collagen type-III glomerulopathy is a rare glomerular disease characterised by the deposition of type III collagen fibres in the subendothelial space and mesangium of the glomerulus. CASE REPORT: Here, we present a case of collagenofibrotic glomerulopathy in a 49-year-old Indian female, the first to be reported from Singapore. Renal biopsy showed PAS (periodic acid-Schiff), silver and Congo red negative, amorphous extracellular material that expanded mesangial and subendothelial regions. Such materials were strongly positive for anti-collagen III immunofluorescent staining. Under electron microscopy, the mesangial and some subendothelial regions were greatly expanded by abundant collagen fibres which were different from normal collagen III fibres in both appearance and periodicity. DISCUSSION: The availability of past renal biopsies for reference offered insight into disease progression. From the initial diagnosis of focal segmental glomerulosclerosis to eventually collagenofibrotic glomerulopathy over a time span of more than 10 years, this case highlights the gradual accumulation of collagen fibres in the glomeruli before classical features are apparent. It also emphasises the importance of electron microscopy in the diagnosis of this disease.
Subject(s)
Collagen Type III , Kidney Diseases/diagnosis , Kidney Diseases/pathology , Kidney Glomerulus/pathology , Biopsy , Female , Humans , Middle Aged , SingaporeABSTRACT
We have previously reported the one-year outcomes of 16 children with severe proliferative lupus nephritis (LN) who were treated using a multi-targeted induction protocol based on intravenous (IV) pulse methylprednisolone (MP), mycophenolate mofetil (MMF) and cyclosporine (CSA). This study examined the long-term renal outcomes of these 16 children, followed up for a median duration of 9.2 years (range 5.8-14.2 years). Primary treatment outcome was complete renal remission. Secondary outcomes included patient and renal survival as well as relapse-free and event-free survival. All patients achieved complete renal remission within 24 months (median 8.7 months, range 4.0-24.0 months). Comparing clinical and laboratory parameters at induction and last follow-up, respectively, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (25.4 ± 8.7 vs. 0.4 ± 0.8), serum complement C3 (47 ± 21 vs. 107 ± 27 mg/dL), estimated glomerular filtration rate (eGFR) (72 ± 57 vs. 109.7 ± 43 ml/min/1.73 m2) and urine protein (6.97 ± 7.09 vs. 0.2 ± 0.02 g/day/1.73 m2) improved significantly (p < 0.05). Kaplan-Meier survival analysis showed a cumulative ten-year renal relapse-free survival of 73.3% when considering relapses with severe proteinuria >1 g/day/1.73 m2. Cumulative probability that hospitalization would not be required was 93.8% at one year, and 71.4% at ten years. Our multi-targeted protocol for induction and maintenance therapy in Asian children with severe proliferative LN resulted in good long-term patient survival and renal preservation, with a good safety profile.
Subject(s)
Cyclosporine/administration & dosage , Immunosuppressive Agents/administration & dosage , Lupus Nephritis/drug therapy , Methylprednisolone/administration & dosage , Mycophenolic Acid/administration & dosage , Administration, Intravenous , Adolescent , Age Factors , Child , Child, Preschool , Disease-Free Survival , Drug Therapy, Combination , Female , Follow-Up Studies , Hospitalization , Humans , Kaplan-Meier Estimate , Logistic Models , Lupus Nephritis/diagnosis , Lupus Nephritis/immunology , Male , Pulse Therapy, Drug , Recurrence , Remission Induction , Risk Factors , Severity of Illness Index , Singapore , Time Factors , Treatment OutcomeABSTRACT
Subdural haematomata (SDH) are usually traumatic in aetiology. Non-traumatic instances of SDH are uncommon, and can rarely be due to metastases involving the dura. Computed tomography or magnetic resonance imaging can be misleading, as the underlying aetiology may be masked by the SDH, or the appearance can simulate meningiomas. A high index of suspicion for SDH is thus required. Under such circumstances, when no overt cause is identified, dural tissue should be sent for histological analysis and blood clot for cytology, even if the appearances are grossly normal at surgery. We present a rare case of a 42-year-old woman who was previously well, but presented with progressive weakness due to acute spontaneous SDH. She required repeated surgical evacuations for SDH and for subsequent recurrent extradural haematomata. After extensive investigations, the cause was identified to be secondary dural metastases from a primary lung carcinoma.
