Treatment with Mycophenolat Mofetil of Steroid-Dependent Asthma—One Case of Severe Asthma

Background. Some patients with severe nonallergic asthma can be difficult to treat with conventional therapy. Mycophenolat Mofetil (MMF) is an immunosuppressive drug with multiple mechanisms. There is theoretical support of specific effect of MMF on severe asthma, in “difficult to treat” patients. The aim of the present case was to explore whether MMF had an effect in one case of severe refractory asthma. The patient. This case deals with one patient with very severe nonallergic treatment refractory asthma who experienced treatment failure on ordinary antiasthmatic treatment and severe adverse events to conventional immunosupressive treatment. She was then treated with MMF. Results. The patient experienced a gain in FEV1 and a reduction in the need for oral glucocorticosteroids as well as seldom need of when needed bronchodilator both during daytime and night. It therefore seems very interesting to examine the use of MMF for severe refractory asthma with further clinical studies and basic cellular trials

LIMPRINT: prevalence of chronic edema in health services in Copenhagen, Denmark

Background: The International Lymphedema Framework developed an international study, Lymphedema Impact and Prevalence International (LIMPRINT) to estimate the prevalence and impact of chronic edema in heterogeneous populations. Methods and results: A validation study using the LIMPRINT methodology was undertaken in Denmark. Participants with CO were identified from in-patient services and compared to those identified within a specialist lymphoedema service and 3 primary care settings. Of 452 inpatients available for screening CO was present in 177(39%) and absent in 275(61%). In addition, 723 participants were found from specialist and primary care services (LPCS). In-patients were significantly older and more likely to be underweight or normal weight. They were more likely to suffer from heart failure/IHD (44.6% vs 23.4%, p<0.001) and have neurological problems (18.1% vs 10.9% p=0.009). The inpatient group were nearly all suffering from secondary Lymphoedema and were less likely to have a cancer or venous diagnosis, but more likely to have immobility as the cause of CO (44.0% vs 17.7%, p<0.001). No in-patients had midline CO compared to 30 within LPCS. Fewer in the in-patient group had standard CO treatment (17.1% vs 73.5%, p<0.001) and subjective control of swelling was worse (19.9% vs 66.7%, p<0.001). Whilst the in-patient group experienced fewer acute infections, when they did so, they were more likely to be admitted to hospital for this (78.6% vs 51.0%, p=0.049). Conclusion: The prevalence of CO in inpatient facilities is high and those with CO have multiple comorbidities that vary according to setting. The feasibility study showed the methodology could be adapted for use in different health systems

LIMPRINT in specialist lymphedema services in United Kingdom, France, Italy and Turkey

Background: There is no standardized international model for specialist lymphoedema services, which covers the types of lymphoedema treated and the treatments provided. The aim of this study was to provide a profile of patients attending specialist lymphoedema services in different countries to explore similarities and differences. Methods and Results: The LIMPRINT core tool was used in specialist lymphoedema services in the UK, France, Italy and Turkey. Services in Turkey saw a slightly younger age group with a higher proportion of female patients reflecting a particular focus on breast cancer-related lymphoedema. There were higher levels of obesity and restricted mobility in patients in the UK compared with other countries. Italy and France saw the highest percentage of patients with primary lymphoedema. Diabetes was a common comorbidity in the UK and Turkey. The UK saw the largest number of patients with lower limb lymphoedema. Conclusions: The results show a wide range of complexity of patients treated in specialist lymphoedema services. Some of the differences between countries may reflect different stages in the evolution of specialist lymphoedema services, rather than a true difference in prevalence, with those with 'younger' services treating a high proportion of patients with cancer and those with more established services treating a wider range of different types of lymphoedema including more elderly people with multiple comorbidities

The VASCERN PPL working group patient pathway for primary and paediatric lymphoedema.

Lymphoedema is caused by an imbalance between fluid production and transport by the lymphatic system. This imbalance can be either caused by reduced transport capacity of the lymphatic system or too much fluid production and leads to swelling associated with tissue changes (skin thickening, fat deposition). Its main common complication is the increased risk of developing cellulitis/erysipelas in the affected area, which can worsen the lymphatic function and can be the cause of raised morbidity of the patient if not treated correctly/urgently. The term primary lymphoedema covers a group of rare conditions caused by abnormal functioning and/or development of the lymphatic system. It covers a highly heterogeneous group of conditions. An accurate diagnosis of primary lymphoedema is crucial for the implementation of an optimal treatment plan and management, as well as to reduce the risk of worsening. Patient care is diverse across Europe, and national specialised centres and networks are not available everywhere. The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) gathers the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular diseases. There are six different working groups in VASCERN, which focus on arterial diseases, hereditary haemorrhagic telangiectasia, neurovascular diseases, lymphoedema and vascular anomalies. The working group Paediatric and Primary Lymphedema (PPL WG) gathers and shares knowledge and expertise in the diagnosis and management of adults and children with primary and paediatric lymphoedema. The members of PPL WG have worked together to produce this opinion statement reflecting strategies on how to approach patients with primary and paediatric lymphoedema. The objective of this patient pathway is to improve patient care by reducing the time to diagnosis, define the best management and follow-up strategies and avoid overuse of resources. Therefore, the patient pathway describes the clinical evaluation and investigations that lead to a clinical diagnosis, the genetic testing, differential diagnosis, the management and treatment options and the patient follow up at expert and local centres. Also, the importance of the patient group participation in the PPL WG is discussed

Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO Registry

OBJECTIVES: The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc). METHODS: The DUO Registry is a European, prospective, multicentre, observational, registry of SSc patients with ongoing digital ulcer disease, irrespective of treatment regimen. Data collected included demographics, SSc duration, SSc subset, internal organ manifestations, autoantibodies, previous and ongoing interventions and complications related to digital ulcers. RESULTS: Up to 19 November 2010 a total of 2439 patients had enrolled into the registry. Most were classified as either limited cutaneous SSc (lcSSc; 52.2%) or diffuse cutaneous SSc (dcSSc; 36.9%). Digital ulcers developed earlier in patients with dcSSc compared with lcSSc. Almost all patients (95.7%) tested positive for antinuclear antibodies, 45.2% for anti-scleroderma-70 and 43.6% for anticentromere antibodies (ACA). The first digital ulcer in the anti-scleroderma-70-positive patient cohort occurred approximately 5 years earlier than the ACA-positive patient group. CONCLUSIONS: This study provides data from a large cohort of SSc patients with a history of digital ulcers. The early occurrence and high frequency of digital ulcer complications are especially seen in patients with dcSSc and/or anti-scleroderma-70 antibodies