Eosinophilic fasciitis is an uncommon disorder of unknown aetiology and poorly-understood pathogenesis. Since 1974, over 250 cases of eosinophilic fasciitis have been reported worldwide. The first case of eosinophilic fasciitis from Bangladesh is reported here. The challenges of diagnosis, treatment, and follow-up, including family and social support, are discussed.
Objectives This study aimed to compare the efficacy of low‐dose prednisolone with conventional high‐dose regimen in proliferative lupus nephritis (LN) for remission. Methods This open‐label randomized clinical trial was conducted in the Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. A total of 32 LN patients were randomized into low‐dose (experimental) and high‐dose (control) groups. All subjects received standard dose of intravenous (I/V) methylprednisolone and pulse I/V cyclophosphamide. Oral prednisolone, 0.5 mg/kg/d and 1 mg/kg/d were given to experimental and control groups respectively for initially 4 weeks then tapered. The patients were followed for 24 weeks. The rates of renal remission (complete and partial) were assessed at 24 weeks. The disease activity, biochemical markers, and quality of life were evaluated at baseline and at 24 weeks. Results Complete renal remission was achieved by 66.7% of patients in each group (P = .99). Renal remission (partial/complete) was achieved by 86.7% and 83.3% of patients in the prednisolone low‐dose group and high‐dose group respectively (P = .99). In between groups, no significant difference was observed in the improvement of active urinary sediments, serum creatinine level, anti‐double‐stranded DNA level, complements level, disease activity and Short Form‐12 score. The prednisolone dose‐related adverse events like cushingoid facies, abdominal stria, infections and serious adverse events like death occurred more in the high‐dose prednisolone group. Conclusions It has been observed that low‐dose prednisolone regimen may be effective in LN. Steroid dose‐related side effects and rate of infections were lower in this group.
Introduction:Rheumatoid arthritis is the most common cause of inflammatory polyarthritis in adults. 1 Many studies suggest that RA involves a combination of genetic factors, including genetic markers as well as familial transmission and environmental factors. The most compelling evidence for a genetic component is in monozygotic twins, in whom the concordance rate is 12% to 15% when one twin is affected compared with 1% for the general population. The risk for a fraternal twin of a patient with RA also is high (about 2% to 5%), but this is not more than the rate for other first-degree relatives. 2,3,4,5 Although the immunogenetics is, at best, incompletely understood, one of the best-studied and perhaps most influential genetic risk factors is the class II MHC haplotype of an individual. Role of HLA-DR in rheumatoid arthritisA genetic link between HLA-DR and RA was initially described in the 1970s with the observation that HLA-DR4 occurred in 70% of RA patients compared with about 30% of controls, giving a relative risk of having RA of approximately 4 to 5 to individuals with HLA-DR4. 6 Many population studies confirmed the original association with DR4, but as Review ArticlesRoles of Genes in the Susceptibility to and Severity of Rheumatoid Arthritis -A Review
Mediastinal lipoma (ML) is a rare entity. Though the mediastinum is the most common site of intrathoracic lipoma, ML constitutes less than 1% of all mediastinal tumours. ML frequently presents on incidental radiographic finding, CT scan is considered the investigation of choice. CT features of lipoma are quite characteristic. They are clinically significant because: (1) Despite their benign nature, these tumours tend to reach an enormous size and can cause compression of lungs and mediastinal structures; (2) It may not always be possible to differentiate a ML from a liposarcoma by CT or MRI alone. DOI: http://dx.doi.org/10.3329/imcj.v6i1.14725 Ibrahim Med. Coll. J. 2012; 6(1): 37-38
Pigmented villonodular synovitis (PVNS) is a rare disorder where abnormal synovial proliferation and insidious swelling are the characteristic features. As insidious joint swelling has many common causes and PVNS is rare entity, so that it may be missed or under evaluated. Here inconclusive biopsy findings at an early stage and features resembling spondyloarthritis (SpA) delayed the diagnosis. A 28-year-old young man presented with insidious swelling of knee. Here repeated aspiration and synovial fluid analysis with routine serological and radiological examination were inconclusive. Then he was labeled and treated as spondyloarthritis (SpA) for four years without improvement. After four years of sufferings he admitted here and reached the diagnosis of PVNS with the help of MRI and synovial biopsy. Sometimes rare disease diagnosis is complicated by the absence of typical features and inconclusive reports. Common differentials may mimic the diagnosis and rare disease may loss the attention. Here PVNS was treated as SpA for four years before being diagnosed. Bangladesh Med J. 2017 May; 46 (2): 53-55
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