Rituximab-induced neutropenia in a patient with inflammatory myopathy and systemic sclerosis overlap disease.

Rituximab (RTX) is a monoclonal chimeric antibody directed against the CD20 antigen of B lymphocytes. Late onset neutropenia (LON) is a recognised complication of rituximab usually occurring 4 weeks after the last dose and is reported in both haematological and rheumatological conditions. However, it has never been described in a patient with myositis and systemic sclerosis overlap disease. We describe a case of LON in a 54-year-old man who was diagnosed with myositis and then systemic sclerosis overlap disease. It resolved within 7 days, and the patient did not suffer neutropenic sepsis or any other complications. We propose similar mechanisms for LON as described in other conditions and routine blood monitoring in such patients.

Coexistent digital gouty and infective flexor tenosynovitis.

Flexor tenosynovitis of the hand is often caused by trauma or infection. Gouty tenosynovitis is an uncommon presentation of the condition and is usually misdiagnosed as infection with the patient undergoing surgery. The coexistence of infection and gout causing flexor tenosynovitis has never been described before in the literature; we report the first ever case and emphasise the importance of its awareness for optimal treatment. A 54-year-old man was initially diagnosed and treated as having infective flexor tenosynovitis and, later, due to a lack of improvement in his symptoms, was discovered to also have gout. We review the literature and suggest management strategy for use in daily clinical practice, including an algorithm, for this presentation.

Differential diagnosis of critical digital ischemia in systemic sclerosis: Report of five cases and review of the literature.

OBJECTIVES: Critical digital ischemia is a rare, but serious complication of systemic sclerosis (SSc) and is not always due solely to the non-inflammatory angiopathy that characterizes the SSc disease process. Our objective was to illustrate the range of presentations and causes of critical digital ischemia in patients with SSc in order to highlight how optimal management is dependent upon establishing the correct diagnosis. METHODS: Five cases exemplifying differential diagnoses were identified and their case notes reviewed in order to extract clinically relevant data and images. A review of the literature was performed in PubMed in English. RESULTS: Causes of critical digital ischemia included typical micro-angiopathic changes and proximal (large vessel) disease. One case highlighted the difficulty of ascertaining whether an inflammatory cause is also present in SSc/SLE overlap syndrome. Two cases demonstrated embolic causes (thromboembolism due to atrial fibrillation and septic emboli). CONCLUSIONS: Critical digital ischemia in patients with SSc requires thorough investigation in order to avoid missing additional potentially modifiable causes including large vessel disease, inflammation, embolism, infection, and paraneoplastic syndromes. A firm evidence base for current medical and surgical interventions is lacking, highlighting the need for further research into the optimum management of this rare, but painful, debilitating, and limb-threatening complication of SSc.

Haemoglobinopathies and the rheumatologist.

The haemoglobinopathies are a relatively common, heterogeneous group of inherited conditions that are the result of either a quantitative abnormality (e.g. thalassaemia) or structural [e.g. sickle cell anaemia (SCA)] of the globin part of the haemoglobin molecule. Musculoskeletal (MSK) complications are common in patients with haemoglobinopathies and may affect the whole of the MSK system, in addition to bone, which is the primary site of the disease. Typical MSK complications include painful vaso-occlusive disease and osteomyelitis in SCA and reduced BMD in thalassaemia. Patients may also develop a number of related (e.g. gout) or unrelated rheumatic diseases (e.g. inflammatory arthritis and autoimmune CTDs). Treatment of MSK conditions in patients with haemoglobinopathies may be challenging (e.g. bone marrow suppression from disease-modifying agents) and in particular in SCA, steroid therapy (by any route) may precipitate potentially severe vaso-occlusive complications. Rheumatologists need to be aware of the range of MSK complications, treatment challenges and the need for such patients to be managed as part of a dedicated multidisciplinary team alongside haematology.

Carotid sinus syndrome is common in very elderly patients undergoing tilt table testing and carotid sinus massage because of syncope or unexplained falls.

BACKGROUND AND AIMS: Although the incidence of falls and syncope increase with age, the underlying mechanisms are not fully understood, particularly in very old patients. We report diagnostic outcomes of tilt table and carotid sinus massage (TT/CSM) testing in a population of older old subjects (82% over 80 years of age) referred for TT/CSM from a falls clinic for assessment of syncope or unexplained falls. METHODS: Prospective observational study between January 1, 2001 and January 1, 2005 involving 290 consecutive subjects undergoing TT/CSM testing according to European Society of Cardiology guidelines for the diagnosis and management of syncope. RESULTS: Combination of TT/CSM provided a positive result in 62% of subjects, and was significantly more likely to be positive in subjects over the age of 80 (68% vs 50%, p=0.001). Carotid sinus syndrome (CSS) was the most common diagnosis, and was significantly more common in subjects over 80 (48% vs 34%, p=0.022) particularly due to a higher incidence of mixed CSS (16% vs 7%, p=0.023). There was no significant difference in the diagnosis of subjects referred due to falls and those reporting syncope (p=0.93). No cardiovascular or neurological adverse events occurred. CONCLUSIONS: In our cohort of very elderly patients, the diagnostic accuracy of tilt testing and carotid sinus massage was high and adverse incidents absent when testing was indicated by a geriatrician experienced in the assessment of falls and syncope. We advocate the use of TT/CSM testing as part of a comprehensive falls/syncope assessment for the diagnostic evaluation of older patients presenting with unexplained falls and/or syncope in whom structured assessment has not identified a cause for their symptoms.

Valvuloplasty for tricuspid stenosis caused by a ventriculoatrial shunt.

A 45-year-old lady developed significant symptomatic tricuspid valve stenosis due to a ventriculoatrial (VA) shunt, implanted in childhood for idiopathic hydrocephalus. This appeared to be a rare complication of a VA shunt. After detailed discussions in multidisciplinary meeting, she underwent successful treatment with a single balloon valvuloplasty. To date, she has not suffered any complications.