Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus.

This case report aims to highlight the importance of keeping catastrophic antiphospholipid syndrome (CAPS) high on the list of differentials in patients with lupus who present with digital ischemia and to understand the workup and treatment of the disease. Catastrophic antiphospholipid syndrome is a life-threatening variant of antiphospholipid syndrome (APS), and it is distinguished on the APS spectrum by its increased intensity and extent of thrombotic outcomes. Less than 1% of patients with APS develop CAPS and the demographic of patients affected are primarily females, 37 ± 14 years old, and have underlying primary APS or systemic lupus erythematosus (SLE). This is the case of a young female with lupus and end-stage renal disease secondary to lupus nephritis who presented to the emergency department for shortness of breath and bilateral leg swelling that eventually progressed to catastrophic antiphospholipid syndrome. She developed pulmonary embolisms, axillary hematoma, and bilateral lower extremity digital gangrene. The treatment course consisted of anticoagulation, steroids, intravenous immunoglobulin (IVIG), above-knee amputation, and eventually rituximab. Diagnosis and treatment of digital ischemia can be complex, especially, in the setting of lupus where the differential diagnosis is broad. A high index of suspicion for CAPS is essential for early diagnosis and treatment.

A Diagnostic Dilemma: Catastrophic or Seronegative Antiphospholipid Syndrome.

Catastrophic antiphospholipid syndrome (CAPS) and seronegative APS (SN-APS) are rare and severe variants of antiphospholipid syndrome (APS). Due to the significant morbidity and mortality associated with these variants, early recognition and adequate treatment with immunomodulatory agents and anticoagulation are crucial. Here, we report a rare presentation of seronegative CAPS in a young adult with systemic lupus erythematosus (SLE) who presented with seizures, encephalopathy, and quadriplegia. Brain imaging revealed intracranial hemorrhage and attenuated vessels in the Circle of Willis suggestive of vasculitis. Imaging also revealed bilateral pulmonary emboli involving the main pulmonary, segmental, and subsegmental arteries; lower extremity deep vein thrombosis in the right common femoral vein; and superficial venous thrombi in the left cephalic and basilic veins. Due to the absence of APS seropositivity and the catastrophic nature of her presentation, namely the widespread thrombi formation and multiorgan involvement, there was high suspicion for a diagnosis of seronegative CAPS. After two weeks of high doses of immunomodulatory agents, plasmapheresis, and intravenous immune globulin (IVIG) treatment, the patient showed clinical improvement and a reduced burden of venous thrombi. The predicament of not being able to use anticoagulation in this patient due to cerebral hemorrhage added to the complexity and uniqueness of this case.

Epidemiology of musculoskeletal complaints and diseases in Qatar: A cross-sectional study.

BACKGROUND: Musculoskeletal (MSK) conditions are considered a significant public health problem on account of their high prevalence in communities worldwide and their pervasive impact. Knowledge of the epidemiology of MSK symptoms and diseases is lacking in Qatar. Obtaining this information will guide local health policymakers in the future strategic planning of the health budget. OBJECTIVE: To estimate the prevalence rate of MSK disorders in the Qatari population above 15 years of age using the Community Oriented Program for the Control of Rheumatic Disease (COPCORD) survey. METHODS: This cross-sectional study targeted 1000 Qatari participants, including 500 males and 500 females. A door-to-door survey was conducted using the Arabic version of the COPCORD questionnaire with the help of research assistants. Participants with positive surveys were asked to visit Hamad General Hospital Rheumatology outpatient clinics for clinical evaluation by a rheumatologist. When necessary, laboratory testing and X-rays were conducted to confirm any MSK diagnosis. RESULTS: A total of 1239 (males, 50.8%) Qatari individuals randomly selected from the different municipalities of Qatar completed the COPCORD survey. Among the participants, 563 (45.4%) screened positive for MSK pain. Knee pain (24.5%) and back pain (23.3%) were the most common sites of pain, and both conditions showed no gender predominance (p = 0.073 and 0.108, respectively). Shoulder, wrist, hand, hip, and neck pain were significantly predominant in females (p < 0.001 for all). A total of 237 MSK disorders were diagnosed in 196 (15.8%) participants, including 181 degenerative joint diseases, 52 soft-tissue rheumatism conditions, and 4 autoimmune inflammatory disorders. Among degenerative joint diseases, knee osteoarthritis (6.4%) was the most common. Among soft-tissue rheumatic conditions, muscular lower back pain (1.9%), myofascial neck pain (0.64%), generalized body pain (0.32%), and shoulder tendinitis (0.7%) were the most common diseases. The autoimmune inflammatory disorders identified included rheumatoid arthritis (n = 2), connective tissue disease (n = 1), and inflammatory bowel disease-associated arthritis (n = 1). CONCLUSION: The overall prevalence rate of MSK disorders in this small cross-sectional cohort of Qatari individuals was 15.8%. Knee pain (24.5%) and knee osteoarthritis (6.4%) were the most common MSK complaints and diagnosis in the studied Qatari population. This study guides future efforts directed toward the prevention and management of MSK diseases. Further studies with a larger sample size are needed to verify the findings.

A Case of Trauma-Induced Arterial Thrombosis Mimicking Vasculitis.

Vasculitis mimickers are structural or pathologic entities that resemble the vasculitis clinical presentation and/or diagnostic findings. Their presence can be a conundrum, and physicians require careful assessment and adequate knowledge physicians when considering a diagnosis of vasculitis. Although they are considered mimickers, the therapeutic approach for most of them differs widely from that of vasculitis as high-dose steroids and potent immunosuppressive regimens are usually indicated in the latter. In fact, steroid therapy is contraindicated and is considered harmful in some of these mimickers (e.g. segmental arterial mediolysis). Therefore, it is important to distinguish them from vasculitis to prevent complications from immunosuppressive therapy. Hereby, we present a challenging case of a 64-year-old man who presented with acute gangrenous changes on his right fingers due to arterial thrombus after trauma resembling vasculitis.

Diplopia as the initial symptom of multiple myeloma in a patient with sarcoidosis.

We present the case of a 45-year-old man with a known history of sarcoidosis who presented with double vision and headache. On examination, he was found to have left abducens and hypoglossal nerve palsy. CT and then MRI demonstrated extensive osseous lesions with a large expansile mass involving the clivus bone and sphenoid sinus. Laboratory data were remarkable for normocytic anaemia, low anion gap and elevated total protein which raised the suspicion for multiple myeloma. Subsequent protein electrophoresis and immunofixation illustrated monoclonal spike of IgG lambda present in the gamma zone. This was followed by a bone marrow biopsy that demonstrated plasma cells compromising around 80% of marrow cellularity. Left sphenoidal mass biopsy was consistent with plasmacytoma. Based on these findings, the patient was initially started on palliative radiation to shrink the intracranial tumour and is currently undergoing induction chemotherapy.

An assessment of the current treatment landscape for rheumatology patients in Qatar: Recognising unmet needs and moving towards solutions.

Objective This study assessed the mode of application (oral, intravenous or subcutaneous (SC)) currently employed in the treatment of rheumatoid arthritis (RA) in patients from Qatar in comparison with patients' individual preferences for the mode of application of their treatment. Methods This study included 294 RA patients visiting three clinics at the main referral hospital in Qatar who were interviewed using a standard questionnaire to determine their preference of mode of application for their disease-modifying antirheumatic drug (DMARD) treatment in relation to their currently employed mode of application. Results The majority of patients were female (76%), and 93% of male patients and 61% of female patients in the study clinics were of a nationality other than Qatari. The highest patient preference recorded was for an oral therapy (69%), compared with injection (23%) and intravenous (8%) therapy. In total, 85% of patients expressed a preference to remain on oral therapy compared with 63% and 58% of intravenous and SC injection patients indicating a preference to remain on their current method of administration. Conclusions This high preference for oral therapies highlights the considerable need for incorporation of new oral targeted synthetic DMARD therapies into clinical practice within the region.

Streptococcus agalactiae Septic Arthritis of the Shoulder and the Sacroiliac Joints: A Case Report.

Invasive group beta-streptococcal arthritis is being increasingly diagnosed as suggested by recent data. We report a case of a middle-aged lady from Sri Lanka who developed septic arthritis of the right shoulder and the left sacroiliac joint as well as an iliopsoas collection caused by Streptococcus agalactiae shortly after labor at Hamad General Hospital in Doha, Qatar. We conclude that Streptococcus agalactiae septic arthritis is rare. It can present with invasive disease in adults. It usually targets older females and immuno compromised patients especially those with risk factors for bacteraemia. Therefore a high index of suspicion is needed. Shoulder and sacroiliac joint affection is not uncommon for unknown reasons. Utilizing imaging modalities such as ultrasonography and magnetic resonance imaging is helpful.

Vitamin D status in health care professionals in Qatar.

OBJECTIVE: To investigate the prevalence of vitamin D deficiency among health care professionals working at Hamad Medical Corporation in Doha, Qatar. METHODS: Between 15th January 2007 and 15th January 2008, 340 healthy volunteers were included in this study. Each subject completed a diary to determine the duration of sunlight exposure, and vitamin D supplements. Serum levels of 25-hydroxyvitamin D (25OHD), parathyroid hormone (PTH), calcium, phosphorus, alkaline phosphatase, total protein, and albumin were obtained. Those with abnormal results were called for counselling. RESULTS: The mean overall vitamin D level was 11.7 ng/ml. It was lower in females (10.3 ng/ml) than in males (13.7 ng/ml). Ninety-seven percent of all participants had a mean level <30 ng/ml. Eighty-seven percent had a mean level of <20 ng/ml. CONCLUSION: We concluded that the prevalence of vitamin D deficiency among health care professionals in Qatar is very high.

Poststreptococcal reactive arthritis and the association with tendonitis, tenosynovitis, and enthesitis.

AIM: To study the clinical presentation of poststreptococcal reactive arthritis (PSRA) and its periarticular manifestation. METHODS: This is a retrospective study. The files of all patients diagnosed with PSRA between January 2004 and November 2007 were reviewed with a predetermined checklist. Patients were included if they met our study criteria for diagnosis of PSRA. RESULTS: A total of 33 files were reviewed; 26 of these patients (14 female, 12 male, Arab and Asian, aged 11-41 years) met our agreed protocol for the diagnosis of PSRA. The ethnic backgrounds of the patients were as follows: 18 patients were from Arab origins and 8 patients were Asians. Twenty-one patients (80%) had asymmetric complaints, whereas 5 patients (20%) had symmetrical complaints. Two patients (7.6%) had monoarthritis, 8 patients (30.76%) had oligoarthritis, and 11 patients (42.3%) had polyarthritis. Five patients (19.23%) had only polytendonitis, tenosynovitis, and/or enthesitis. Nine patients (34.61%) had tendonitis, tenosynovitis, or enthesitis alone or with arthritis/arthralgia. The average elevation of antistreptolysin antibodies titer was 624.8 and the average sedimentation rate 44 mm/H. The response to nonsteroidal antiinflammatory drugs was generally good (84.6%), being poor in only 4 patients (15.38%) who required treatment with corticosteroids. Prophylactic penicillin was given to 15 patients (57%). No patient had carditis on presentation or follow-up. CONCLUSIONS: It is concluded that polytendonitis, tenosynovitis, and enthesitis are common presentations in PSRA and could be the only manifestation of poststreptococcal infection.