Polyarthritis as the Initial Presentation of Hyper Eosinophilic Syndrome.

Hyper eosinophilic syndrome (HES) is a rare condition with a potential for morbidity and mortality, if left untreated. Therefore, it is important to highlight it, as often these cases are misdiagnosed and mismanaged, specially when presenting with an atypical initial presentation. This case report describes an unusual initial clinical presentation of HES. Patient was a 75-year lady presenting to Rheumatology Clinic with short duration of fever and polyarthritis. Joint aspiration showed purulent fluid with a cell count of 61,000/mm3 with predominant neutrophils and eosinophils. Her peripheral blood also showed a high white blood cell (WBC) count (80,700/mm3 with 73% eosinophils). Her workup for eosinophilic leukemia was negative, so a diagnosis of HES was made. She was initiated on corticosteroids and hydroxycarbamide as first-line therapy. Unfortunately, the patient was unresponsive to steroids with her WBC count rising to 130,000/mm3 and her clinical course was complicated by cardiac failure and peripheral neuropathy. Improvement in arthritis and peripheral eosinophilia was noted after she was given imatinib and stabilised clinically.

Axial Spondyloarthritis In Patients With Chronic Backache Using Assessment Of Spondyloarthritis International Society Criteria For Axial Spondyloarthritis.

BACKGROUND: Low back pain (LBP) is considered as one of the most frequent health problems which is responsible for forming a huge worldwide burden. This study was conducted with the aim to determine the frequency of axial-Spondyloarthropathy (axSpA) in patients presenting with chronic backache using Assessment of Spondyloarthritis International Society (ASAS) Criteria for axSpA. METHODS: A total of 231 participants of either gender were enrolled with complaint of backache of more than or equal to 3 months duration and younger than 45 years. In the first stage, patients were interviewed and examined using standard questionnaire. In the second stage after going through laboratory investigations and imaging patients were classified into either axSpA (those meeting ASAS Criteria for axSpA) or non-SpA. RESULTS: There were 65 males and 166 females. Mean age was 36.26 years. Eighty-nine (39%) patients were found to have axSpA as per ASAS Criteria. Majority of patients 70 (78.6%) in the imaging arm of ASAS Criteria were picked via MRI of sacroiliac joints. Peripheral arthritis was seen in 57 (64%) and Enthesitis in 52 (58.4%). CONCLUSIONS: MRI performed exceptionally to reveal sacroiliitis highlighting the importance of this imaging modality in axSpA, which we recommend to be included in diagnostic algorithm in evaluating patients with chronic backache under 45 years age.

Relationship of anthropometric measures with Knee Osteoarthritis in diabetes mellitus.

OBJECTIVE: To determine the frequency of knee osteoarthritis in adult patients with Diabetes mellitus and its association with body mass index (BMI) in kg/m2 and waist circumference (WC). METHODS: This is a cross-sectional comparative study at a tertiary hospital based in an industrial area of Karachi. Patient population comprised of all adult diabetic subjects. Clinical and demographic data was obtained with detailed musculoskeletal examination on all patients. BMI and WC were measured as kg/m2 and cm respectively. Data was analyzed on SPSS version 15. RESULTS: A total of 413 subjects were recruited. Among them diabetic and non-diabetic subjects were 210 and 203 respectively. Mean age of diabetics was 50.7+-10.2 years as compared to non-diabetic subjects i.e. 49.5+-10.5 years. Proportion of male subjects was 72(34.3%) and 71(35.0) respectively in both groups. Mean duration of diabetes was 6.2 years. Frequency of knee osteoarthritis (OA) was found to be 52(24.8%) and 54(26.6%) in diabetic and non-diabetic respectively. Among the diabetic group 6 (18%) subjects with OA had normal BMI (18.5-22.9) whereas 4 (16%) were overweight (BMI 23-24.9) and 41(27.5%) were obese(BMI ≥25). Near 98% (n=51) of the diabetic patients with OA had high waist circumference. Females (n= 42, 31.1%) were more frequent than males (n=9, 16%) in the diabetic subjects with OA and a higher WC. CONCLUSION: Both diabetic and non diabetic group did not show any difference in the frequency of knee OA. However, frequency of knee OA showed a significant difference between overweight and obese category of BMI.WC appears as a strong predictor of knee osteoarthritis.

LOW BONE MINERAL DENSITY AMONG PATIENTS WITH NEWLY DIAGNOSED RHEUMATOID ARTHRITIS.

BACKGROUND: Osteoporosis is an early and common feature in rheumatoid arthritis. Apart from other manifestations, Osteoporosis is an extra-articular manifestation of rheumatoid arthritis whichmay result in increased risk of fractures, morbidity mortality, and associated healthcare costs. This study evaluates bone mineral density changes in patients withrheumatoid arthritis of recent-onset. METHODS: This cross sectional descriptive study was conducted in the Rheumatology Department of a tertiary care hospital in Karachi. Data was collected from 76 patients presenting with seropositive or seronegative rheumatoid arthritis. Bone mineral density of these patients measured at lumbar spine and hip by using dual energy x-ray absorptiometrys can. Variables like age, gender, BMI, menstrual status, disease duration, erythrocyte sedimentation rate, vitamin D level, clinical disease activity index and seropositivity for rheumatoid arthritis were measured along with outcome variables. RESULTS: A total of 104 patients fulfilling inclusion criteria were registered with 28 excluded from study. A mong the remaining 76 patients, 68 (89.50%) were female, with mean age of patients (with low bone mineral density) as 50.95 ± 7.87 years. Nineteen (25%) patients had low bone mineral density, 68.52% had low BMD at spine while 10.52% at hip and 21.05% at spine and hip both. Low bone mineral density was found higher in patients with seronegative 7 (50%) as compared to seropositive patients 12 (19.4%) (p-value 0.017), whereas low bone mineral d ensity was found higher 12 (70.6%) among post-menopausal women. CONCLUSION: Low BMD was found in 25% of patients at earlier stage of the rheumatoid arthritis with seropositivity, age and menopausal status as significant risk factors.

Frequency of low bone mineral density in spondyloarthropathy presenting at a tertiary care hospital.

OBJECTIVE: To determine the frequency and risk factors of low bone mineral density in patients with spondyloarthropathies. METHODS: The cross-sectional study was conducted at the Rheumatology Department of a tertiary care hospital in Karachi between June and November 2014, and comprised spondyloarthritis patients whose bone mineral density of lumbar spine and hips was measured using dual energy X-ray absorptiometry scan. Variables like disease duration, diagnosis, human leukocyte antigen subtype B27, erythrocyte sedimentation rate, C-reactive protein, Bath ankylosing spondylitis disease activity Index, Bath ankylosing spondylitis functional index, Bath ankylosing spondylitis metrology index were measured along with outcomes, differentiating between osteopenia and/or osteoporosis. SPSS 21 was used for statistical analysis. RESULTS: Of the 25 patients in the study, 16(64%) were males, 19(76%) had predominant axial involvement, and 20(80%) had duration of disease less than 10 years. Low bone mineral density at the spine and hip was found in 18(72%). Osteopenia was present in 9(36%) at hip and 8(32%)in spine, while osteoporosis was seen in 5(20%) at hip and 9(36%) in the spine. No significant association was found between bone mineral density and all the other parameters measured (p>0.05 each). CONCLUSIONS: Majority of spondyloarthritis patients had decreased bone mineral density which could be observed in early stages of the disease.

The calcium invasion: Calciphylaxis in Lupus.

Calciphylaxis is a poorly understood and highly morbid syndrome of vascular calcification and skin necrosis. We describe the case of a 52-year old woman with systemic lupus erythematosus (SLE), inappropriately treated with oral steroids for 18-years who developed renal impairment followed by skin necrosis and gangrene of right hand. She had subcutaneous calcium deposition with bilateral renal stones and widespread vascular calcifications. She was diagnosed with calciphylaxis and in spite of treatment died of a myocardial infarction.

Comparison of glycaemic response to honey and glucose in type 2 diabetes.

OBJECTIVE: To compare the glycaemic effect of 75 gram and 30 gram of natural honey in a honey tolerance test with that of 75 gram glucose in type 2 diabetics. METHODS: The experimental study was conducted at the Jinnah Medical College Hospital, Karachi, and comprised 97 type 2 diabetic patients who came to the out-patient department between March and August 2011. The patients were randomly divided into 75 gram honey group (group 1), 30 gram honey group (group 2), and 75 gram glucose group (group 3). Fasting blood samples were obtained as well as after 1 and 2 hour. SPSS 11 was used for statistical analysis. RESULTS: Of the 97 participants, 62 (64%) were females and 35 (36%) males.Their ages ranged from 25-68 years. Mean rise in blood glucose after two hours in group 2 was 30 mg/dl; group 1, 85mg/dl, and group 3, 170 mg/dl. The difference was statistically significant (p <0.005).The glucose response was significantly lower at 2 hours in group 2 (p <0.001) compared to group 1 or group 2. A significant difference was also seen in group 1 and 3 (p <0.0001). The plasma glucose level in response to honey peaked at 60 min and showed a rapid decline compared to that of glucose, indicating a lower glycaemic response of honey. A small proportion of patients 3 (10.7%) even showed a glucose lowering effect after low dose of honey. CONCLUSION: Low dose of honey can be a valuable sugar substitute for patients with diabetes.

Lupus, still a mystery: a comparison of clinical features of Pakistani population living in suburbs of Karachi with other Asian countries.

OBJECTIVE: To determine the presenting features of patients with systemic lupus erythematosus at a private hospital in Karachi, and to compare the features with those of other Asian populations. METHODS: The retrospective study comprised records of all lupus cases meeting the revised American Rheumatism Association criteria at the time of presentation at Jinnah Medical College Hospital, Karachi, from May 2008 to June 2011. Demographic and clinical data was analysed using SPSS 11.5. RESULTS: Of the 105 cases in the study, there were 6 (5.7%) males and 99 (94.3%) females, with a male-to-female ratio of 1:16 and a mean age of 31.6+/-10.5 years. Clinical manifestations included: constitutional symptoms in (n=69; 65.7%), arthropathy (n=81; 77%), cutaneous involvement (n=39; 37%), lupus nephritis (n=24; 22.8%), pleurisy (n=9; 8.6%), Raynaud's phenomenon (n=24; 22.8%), and vasculitis (n=18; 17%). One (0.95%) patient presented with mononeuritis multiplex, and 1 (0.95%) with acute pancreatitis. CONCLUSION: The diversity in clinical presentation appeared to be a reflection of the great variability that exists among Asian countries with regards to their genetic, environmental and socio-demographic backgrounds. The differences also existed in our own population, suggesting some unknown etiology.

Interferon-induced central retinal vein thrombosis.

A middle-aged lady presented with sudden onset of unilateral central retinal vein thrombosis after completing 6 months course of interferon and ribavirin for chronic hepatitis C infection. She had no risk factors and all her thrombophilia workup was normal, however, she was found to be dyslipidemic which may have contributed to atherosclerosis and predispose to thrombosis. Despite anticoagulation, her visual acuity deteriorated. This case illustrates the possibility of unpredictable visual complication of interferon. Frequent eye examination should be undertaken in patients having underlying risk factors like diabetes, hypertension or dyslipidemia undergoing interferon therapy.

'The eyes see what the mind knows.' Adult-onset Still's disease, a case series and review in a south Asian population.

AIM: Adult-onset Still's disease (AOSD) is a rare disease. Very few cases have been reported from the South-Asian region so the aim of this study is to assess the clinical and laboratory aspects of 15 patients with AOSD in a tertiary referral hospital in Karachi. METHODS: Retrospective data was collected from all patients diagnosed using Yamaguchi criteria for AOSD between January 2004 and December 2010 at Jinnah Medical College Hospital, Karachi. RESULTS: Data of 15 patients with AOSD were analyzed. Their ages ranged from 17 to 55 years, the male-to-female ratio being 6:1. The most common clinical features were fever and articular symptoms (100%), sore throat (60%), rash (53.3%), weight loss (93.3%), lymphadenopathy (40%) and elevated erythrocyte sedimentation rate (86.7%). All patients had leukocytosis with counts>20,000/mm 3 were seen in 40%. Elevated liver enzymes were present in 80% of the case series and hyperferritinemia in 100% with a mean of 3,962 ng/mL (range 555-13,865). Ambiguity in presentation and lack of serologic markers make diagnosis of AOSD difficult as 40% of patients were receiving empirical anti-tuberculous therapy prior to final diagnosis. CONCLUSION: It is necessary for physicians to have a high index of suspicion for AOSD in patients with high-grade fever, arthralgia and leukocytosis.