Large Variability of Frequency and Type of Physical Therapy in Patients in the German Network for Systemic Sclerosis.

OBJECTIVE: To determine the type and frequency of physical therapy (PT) prescribed by physicians for patients in the registry of the German Network for Systemic Sclerosis. METHODS: The data for 4,252 patients were analyzed using descriptive statistics, chi-square tests, and odds ratios (ORs). RESULTS: Overall, 37.4% of patients (1,590 of 4,252) received PT at the end of a yearly follow-up. The most frequently used type of PT was lymphatic drainage (n = 1,061, 36.8%), followed by exercise therapy (n = 1,047, 36.3%) and heat therapy (n = 689, 23.9%). More than three-fourths of treated patients (82%) received 1 or 2 different forms of PT simultaneously. The prescription of PT was associated with the extent of skin fibrosis as measured by the modified Rodnan skin thickness score (<10 [41.8% of patients], 11-20 [55.8% of patients], and >21 [63.9% of patients]; P < 0.001). Patients with musculoskeletal involvement (e.g., arthritis, muscle weakness, joint contractures, tendon friction rubs) had a higher chance of receiving PT than patients without these symptoms, with corresponding ORs ranging from 1.96 (95% confidence interval [95% CI] 1.69-2.28) for joint contractures to 3.83 (95% CI 2.89-5.08) for arthritis. When comparing the type of PT prescription across the initial and all follow-up visits from 2003 to 2017, significant alterations with a decreasing frequency of patients receiving PT could be observed (P = 0.001). CONCLUSION: To our knowledge, this is the first study reporting the use of PT in patients with systemic sclerosis (SSc) in a large cohort. Although SSc is characterized by considerable disability and restriction of motion, <40% of patients received PT.

[Sepsis with acral necrosis in a patient with Sharp syndrome - case 8/2010]. / Sepsis mit akralen Nekrosen bei einer Patientin mit Sharp-Syndrom - Fall 8/2010.

HISTORY AND ADMISSION FINDINGS: We report on a patient with Sharp-Syndrome who was referred to our emergency department with sepsis. In addition, the patient showed acral necrosis, particularly of the distal phalanges of the hands and of the tip of the nose. INVESTIGATIONS: Laboratory analyses showed an elevation of the inflammatory parameters (C-reactive protein elevation, leukocytosis). Furthermore, procalcitonin and the D-dimers were increased, antithrombin III, however, was decreased. The thoracic/abdominal computed tomography (CT) showed bilateral inferior lobe pneumonia with concomitant pleural effusions. As a secondary diagnostic finding the thoracic/abdominal CT and the abdominal ultrasound showed a markedly reduced size of the spleen. Finally, bacteria (Streptococcus pneumoniae) were found in the blood of the patient. DIAGNOSIS, TREATMENT AND COURSE: We diagnosed pneumococcal sepsis with disseminated intravasal coagulation and acral necrosis caused by pneumonia. The immune status was impaired due to immunosuppressive therapy (prednisolon and azathioprin) and functional asplenism. The patient was stabilized with antibiotic treatment, hydration, and drotrecogin (protein C). Transiently catecholamin treatment and oxygen substitution were necessary. Alprostadil was used to treat acral circulatory disorder. CONCLUSIONS: This case report shows the importance of consequent screening for organ manifestations in autoimmune diseases. In particular, this case report highlights the relevance of prophylactic vaccination in patients with autoimmune diseases, primarily those with autosplenectomy. Furthermore, this article gives a short overview about the pathogenesis, the diagnostic criteria of the Sharp-syndrome. The frequencies of organ involvement and the treatment options are also discussed.

Interferon-alpha--a new therapeutic option in refractory juvenile Behçet's disease with CNS involvement.

OBJECTIVE: To report the successful treatment with recombinant human IFN- alpha 2a (rhIFN-alpha2a) in two male adolescents suffering from severe treatment-resistant Behçet's disease (BD) with central nervous system (CNS) involvement. METHODS: The patients were 14- and 15-yrs old. Both met the International Study Group for Behçet's disease, O'Duffy and the Japanese criteria for the classification or diagnosis of BD. Signs of CNS involvement were impaired sensorimotor function of the left arm, hemiparesis of right arm and leg, dizziness and walking instability in Patient 1, weakness of both legs, impaired bladder-, bowel- and sexual function in Patient 2 and vasculitic lesions on cranial MRI in both patients. RhIFN-alpha2a was administered initially at 3 million IU/day for 4 weeks followed by 3 x 3 million IU/week. RESULTS: Complete remission was achieved in Patient 1 (reduction in BD activity score from 17 to 2). Patient 2 experienced remarkable improvement (reduction of BD activity score from 23 to 15). In both patients the MRI lesions improved. Patient 2 had mild flu-like symptoms as adverse effect. CONCLUSION: RhIFN-alpha2a was effective and well tolerated in these juvenile patients with severe neurological BD. Regarding the serious consequences following ocular and CNS affection and adverse effects of steroid dependency, administration of rhIFN-alpha2a at an earlier time point needs to be considered.

B-cell depletion in patients with rheumatoid arthritis refractant to multiple TNF blockers and the interleukin 1 receptor-antagonist anakinra. Good responses in an extreme negative selection.

To evaluate the efficacy of Rituximab in a negatively selected patient cohort, with inadequate response to different disease modifying drugs (DMARDs) and to at least two biologicals. Fifteen patients with severe rheumatoid arthritis with inefficacy of an average of 5 DMARDS and 2.5 TNF antagonists were treated with Rituximab. Eight patients were ineffectively pretreated with Anakinra as well. The disease activity score (DAS28) and the morning stiffness served for assessment of the clinical response. For maintenance treatment different conventional DMARDs were used (4xMTX, 4xLeflunomide, 1xmycophenolate, 1xsirolimus, 1xhydroxychloroquine). At baseline visit the mean DAS 28 was 5.9. The mean duration of morning stiffness was 99.6 min. At month 6 the mean DAS28 was 3.95. Forty percent (6 patients) showed a good and 33% (5 patients) a moderate response. Morning stiffness improved to 43 min. In this negatively selected group of patients Rituximab was safe and effective.

Rituximab for treatment-resistant extensive Wegener's granulomatosis--additive effects of a maintenance treatment with leflunomide.

Extensive Wegener's granulomatosis (WG) is treated by glucocorticosteroids (GC) and cyclophosphamide (CYC). In some cases, the disease is refractory to CYC. For those patients the depletion of B-lymphocytes with rituximab is a promising new treatment modality. This is a retrospective study of six patients receiving rituximab (RTX) with 4 x 375 mg/m(2) body surface weekly because of inefficacy of CYC. Proteinase-3-antineutrophil cytoplasmic antibodies (PR3-ANCA) and c-ANCAs were assessed. For clinical follow-up the Birmingham Vasculitis Activity Score for WG (BVAS/WG) was used. In five of the six cases, leflunomide (LEF) was given as maintenance treatment. Mean follow up was 16 months (12-21 months). The median PR3-ANCA titer fell from 36.8 U/ml at baseline to 21.4 U/ml after 3 months, 8.3 after 6 months, and 4.3 at month 12. The median BVAS/WG at baseline was 5 and 0 after 1 month. Two minor relapses could be noticed at month 3. After 6 months, one patient still had a BVAS of 1, all the others had a BVAS of 0. At month 18, a major relapse occurred in one patient, which was successfully retreated with RTX. The RTX infusions were well tolerated. Rituximab is a well-tolerated, very effective medication for patients with Wegener's granulomatosis. Leflunomide maintenance may increase the efficacy of rituximab and prolong the disease-free period.

Familial inflammatory Sneddon's syndrome-case report and review of the literature.

Sneddon's syndrome (SNS) which originally was a clinical diagnosis, is now regarded as a common clinical manifestation of different disease entities. It has been divided into idiopathic, autoimmune and thromboembolic subsets or in systemic lupus erythematosus (SLE)-associated, antiphospholipid syndrome (APS)-associated and primary forms. Familial occurrence of Sneddon's syndrome is rare. We present a familial case of Sneddon's syndrome with inflammatory disease pattern, early disease onset and association with autoimmune thyroid disease and anticardiolipin antibodies. Although most authors reporting on adult cases of SNS consider it a non-inflammatory, thromboembolic process, the study of cases with early onset brings attention to the possible inflammatory origin of the syndrome.

[Diagnosis of the antiphospholipid syndrome in anticoagulated patients]. / Diagnose des Antiphospholipid-Syndrom beim antikoagulierten Patient: Was gibt es zu beachten?

The antiphospholipid syndrome is characterized by thromboembolic events and/or recurrent miscarriages in the presence of anticardiolipin antibodies and/or a lupus anticoagulant. Anticardiolipin antibodies are detected by ELISA whereas lupus anticoagulant detection includes a variety of coagulometric tests. However, a large number of patients with suspected antiphospholipid syndrome are anticoagulated with either heparin or coumadin when evaluated for the presence of a lupus anticoagulant and false positive test results may ensue, thus making coagulometric testing unreliable in this situation. Modifications of standard coagulometric tests have been suggested in order to circumvent this problem and studies of several patient cohorts have shown that the majority of patients with the antiphospholipid syndrome are anticardiolipin positive. However diagnosis in patients receiving anticoagulation remains a difficult task.

Autologous stem cell transplantation in the treatment of systemic sclerosis: report from the EBMT/EULAR Registry.

OBJECTIVE: To analyse the durability of the responses after haematopoietic stem cell transplantation (HSCT) for severe systemic sclerosis (SSc) and determine whether the high transplant related mortality (TRM) improved with experience. This EBMT/EULAR report describes the longer outcome of patients originally described in addition to newly recruited cases. METHODS: Only patients with SSc, treated by HSCT in European phase I-II studies from 1996 up to 2002, with more than 6 months of follow up were included. Transplant regimens were according to the international consensus statements. Repeated evaluations analysed complete, partial, or non-response and the probability of disease progression and survival after HSCT (Kaplan-Meier). RESULTS: Given as median (range). Among 57 patients aged 40 (9.1-68.7) years the skin scores improved at 6 (n = 37 patients), 12 (n = 30), 24 (n = 19), and 36 (n = 10) months after HSCT (p<0.005). After 22.9 (4.5-81.1) months, partial (n = 32) or complete response (n = 14) was seen in 92% and non-response in 8% (n = 4) of 50 observed cases. 35% of the patients with initial partial (n = 13/32) or complete response (n = 3/14) relapsed within 10 (2.2-48.7) months after HSCT. The TRM was 8.7% (n = 5/57). Deaths related to progression accounted for 14% (n = 8/57) of the 23% (n = 13/57) total mortality rate. At 5 years, progression probability was 48% (95% CI 28 to 68) and the projected survival was 72% (95% CI 59 to 75). CONCLUSION: This EBMT/EULAR report showed that response in two thirds of the patients after HSCT was durable with an acceptable TRM. Based on these results prospective, randomised trials are proceeding.