Estimation of 10-Year Fracture Risk with and without Bone Mineral Density in Patients with Rheumatoid Arthritis.

Rheumatoid arthritis (RA) is the most common inflammatory arthritis affecting 0.5-1.0% of the general population worldwide and although RA is properly considered a disease of the joints, it can cause a variety of extra-articular manifestations. This study was performed to find out any discrepancy in fracture risk estimates with and without bone mineral density (BMD) in rheumatoid arthritis (RA) patients. This observational cross-sectional study was carried out in the Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Bangladesh from July 2013 to July 2015. Total 65 consecutive patients with RA fulfilling ACR/EULAR criteria aged 40-90 year were recruited. Ten year fracture risk of these patients was evaluated by the FRAX score with and without BMD and differences were observed. FRAX score without BMD revealed that major fracture risk was low in 58(89.2%) patients, moderate in 7(10.8%) patients but re-estimation with BMD revealed that 55(84.6%) patients remained in low risk group, 8(12.3%) patients in moderate risk group and 2(3.1%) patients went to the high risk group. In case of hip fracture risk without BMD, risk was low in 58(89.2%) patients, high in 7(10.8%) patients; but with BMD, 50(76.9%) patients remained in low risk group but risk of 15(23.1%) patients became high. Almost all the high risk patients (93.3%) were ≥55 years of age. Increasing age, female sex, disease duration and use of steroid were positively correlated with increased FRAX score where as high BMI and high BMD were associated with low FRAX score. But in multivariate analysis it was found that only relation with age was at statistically significant level. Significant numbers of patients with rheumatoid arthritis have high risk of fracture especially hip fracture. The mean of FRAX score increased in both major & hip osteoporotic fracture risk after adding BMD. More than half of the patients above fifty five years or more had high risk of fracture. So, BMD should be done in patients aged more than fifty five.

Polyarthritis is a Rare Manifestation of Pachydermoperiostosis: A Case Report.

Pachydermoperiostosis or primary hypertrophic osteoarthropathy, also known as Touraine-Solente-Gole syndrome, is a rare process, frequently inherited. In its complete form it is characterized by pachydermia (thickening of the skin), skeletal changes (periostosis) and acropachia (digital clubbing). Diagnosis can be made considering the typical clinical features and the histological feature. We report a patient of 25-year old man presented with joint pain involving in multiple joints for last 7 years and progressive enlargement of his hands and feet with profuse sweating of palms and soles for last 4 years. Physical examination revealed thickened skin with excessive furrowing of his forehead, dropping of both eyelids, clubbing of all fingers, toes and enlargement of his hands and feet with pamoplantar hyperhidrosis. Laboratory investigation shows raised CRP, X-ray feet lateral view showed normal heel pad thickness, new bone formation and periosteal elevation in lower end of tibia and fibula and skin biopsy showed dense fibrocollagenous tissue in dermis and subcutis and mild acanthosis. With this scenario he was diagnosed as primary hypertrophic osteoarthropathy (Pachydermoperiostosis). After diagnosis he was treated with cholchicine (0.6mg) twice daily, naproxen (500mg) twice daily, and risedronate (150mg) monthly. After one year his skin texture became less thickened, joint pain improved, there was no further enlargement of acral part of fore arm. In this report we review the characteristic features of this syndrome. We highlight the importance of ruling out secondary forms of hypertrophic osteoarthropathy and of a close follow-up of these patients because of complications that might develop on long-term. Although no treatment was satisfactory, we wanted to emphasize that NSAIDs, Cholchicine and risedronate could be an effective treatment option.

Scleredema Diabeticorum - A Case Report.

Buschke Scleredema is a rare connective tissue disorder of unknown aetiology and characterized by thickening of the dermis. It resembles systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. It may be classified into three clinical groups, each has a different history, course, and prognosis. Histology of skin is characterized by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis. Differential diagnosis can be made considering the typical clinical features and the histological peculiarity. No therapy is effective. In this communication we have presented a 54 year old man with scleredema successfully treated by PUVA and methotrexate. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment.

Echocardiographic assessment of cardiac involvement in systemic lupus erythematosus patients.

This study was designed to assess cardiac abnormalities in patients with systemic lupus erythematosus (SLE) by echocardiography. It was an analytic type of cross sectional study, conducted in lupus clinic, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2008 to June 2009. Fifty lupus patients, diagnosed on the basis of ACR criteria, without cardiovascular symptoms, were enrolled in the study and were evaluated by standard echocardiography with color Doppler. SLEDAI was applied for assessment of disease activity. Out of 50 patients 80% had abnormal echocardiographic findings. Pericardial thickening was found in 38% patients, pericardial effusion 20%, diastolic dysfunction 72%, hypokinesia of ventricular wall 8%, overall valvular abnormalities 20%, commonest being aortic regurgitation (12%), followed by mitral regurgitation (8%), and 6% had pulmonary hypertension. Males (100%) were more vulnerable to cardiac involvement than females (68.2%) and later age of disease onset (31-40 years) was associated with higher (87.5%) chance of echo abnormalities. The differences, however, were not statistically significant (p>0.05). There was significant relationship between disease duration and cardiac abnormalities (p<0.01). Active disease (80.08%) was associated with higher frequency of cardiac involvement than disease in remission (62.50%) but the result was not statistically significant (p=0.151). Cardiac abnormalities are very common in lupus patients even when clinically asymptomatic from cardiac aspect. Echocardiography is an excellent non-invasive tool for cardiac evaluation. These observations emphasize a need for further assessment of early intervention to reduce subsequent cardiac morbidity and mortality among the lupus patients.

Wegener's granulomatosis - a case report.

Wegener's granulomatosis is an uncommon multi-systemic disease characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tracts and general focal necrotizing vasculitis (commonly known as Wegener's triad). The diagnosis of Wegener's granulomatosis is suggested from the clinical and laboratory findings and from the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCA) although the absence of ANCA does not exclude the diagnosis. We described a case of 27-year-old man with pansinusitis, mild azotemia and initial respiratory tract symptoms such as foul smelling nasal discharge and epistaxis. Later he developed fever, poly-arthritis, abdominal pain and haematuria. There were multiple painful oral ulcers and skin showed multiple palpable purpuric rash. C-ANCA was positive. He was treated with IV methyl prednisolone 1gm daily for 3 days followed by oral prednisolone 1mg/kg body weight and oral cyclophosphamide. His condition improved dramatically and on follow up after 3 months he was reasonably well. In this report, we wanted to emphasize that Wegener's granulomatosis, although rare, should be considered in the above clinical scenario and treatment should be initiated as soon as possible.