[Ultrasound of nerves of the lower extremities]. / Nervensonographie der unteren Extremitäten.

The ability to visualize the nerves of the lower extremities differs from that of the upper extremities in sonography because the soft tissue cover is significantly larger in some cases. Landmarks are also defined for the lower extremities, which enable precise visualization of the nerves. Nerves and muscles are to be understood as a functional unit. In addition to the clarification of nerve compression syndromes, polyneuropathies and nerve tumors, sonography is also used to visualize muscle atrophy.

Osteoporosis Caused by Systemic Mastocytosis: Prevalence in a Cohort of 8392 Patients with Osteoporosis.

Indolent systemic mastocytosis (ISM) is a group of heterogenous diseases characterized by abnormal accumulation of mast cells in at least one organ. ISM can be a cause of osteoporosis. The aim of this study is to determine the prevalence, and the prognosis of ISM in a cohort of patients with osteoporosis. In this monocentric and retrospective study, patients with osteoporosis who did not receive a bone biopsy (cohort 1) and patients that subsequently received a diagnostic bone biopsy for differential diagnosis (cohort 2) are compared with patients who are diagnosed with ISM (cohort 3). A total of 8392 patients are diagnosed with osteoporosis. Out of these patients 1374 underwent a diagnostic bone biopsy resulting in 43 patients with ISM. These figures indicate that ISM is diagnosed in 0.5% of patients with osteoporosis and in 3.1% (men 5.8%) of patients who underwent bone biopsies. Patients with ISM sustained significantly more vertebral fractures in comparison to patients in cohort 2 (4.4 ± 3.6 versus 2.4 ± 2.5 vertebral fractures, p < 0.001) and women were significantly younger compared to cohort 2 (57.3 ± 12 versus 63.6 ± 12 years, p < 0.05). Only 33% showed an involvement of the skin (urticaria pigmentosa). ISM is a rare cause of osteoporosis (0.5%). However, in a subgroup of rather young male patients with osteoporosis the prevalence is more than 5%. Thus, ISM should be considered in premenopausal women and men presenting with vertebral fractures even if urticaria pigmentosa is not present.

Long-term outcome of patients with pregnancy and lactation-associated osteoporosis (PLO) with a particular focus on quality of life.

BACKGROUND: Pregnancy and lactation-associated osteoporosis (PLO) is a very rare form of osteoporosis. Vertebral fractures either occur in the last trimester of pregnancy or after childbirth. Pathogenesis is still unclear. Until recently, almost no data existed in regards to the quality of life and long-term clinical outcome of patients with PLO. OBJECTIVES: To determine the long-term clinical outcome of patients with pregnancy and lactation-associated osteoporosis (PLO) with respect to the following factors: pain, quality of life, mental condition, vertebral fractures, and capacity to work. METHODS: In this single-center retrospective study, patients were reviewed for at least 2 years, more than 50% of them were followed up until menopause. Bone density (DXA) and vertebral fractures were assessed. Standardized questionnaires were used to analyze factors such as quality of life (Qualeffo-41), anxiety and depression (PHQ-4), and pain (the visual analog scale [VAS]). Additionally, a questionnaire was designed in order to evaluate and discuss some of the reasons behind the occurrence of mental distress at the onset of symptoms. RESULTS: Our report shows the clinical course of 20 patients with PLO, 11 of them until menopause (on average 16.3 years after onset of symptoms). When diagnosis was made, 5.4 vertebral fractures were noticed on average. Three of the 20 patients developed subsequent fractures in the following years. The diagnosis of PLO was made on average after 3.3 months after the onset of symptoms. At the beginning of the investigation, physical and mental health of all patients were poor, but improved within the first 2 years and continued doing so until menopause. The average time it took to return to employment was 3.3 years. CONCLUSION: PLO has a significant impact on pain, mental state, quality of life, and capacity to work. However, the long-term prognosis is promising. The severe mental distress is presumably related to several contributing factors in life such as physical integrity and independence, family life, employment, and financial security.Key Points• PLO has a strong impact on quality of life and can lead to severe mental distress.• At onset of symptoms, patients with PLO are in very poor mental and physical condition; however, the long-term outcome after inpatient rehabilitation seems to be good.• Most patients do not suffer subsequent vertebral fractures until the menopause.

Characteristics of episodic breathlessness as reported by patients with advanced chronic obstructive pulmonary disease and lung cancer: Results of a descriptive cohort study.

BACKGROUND: Episodic breathlessness is one form of refractory breathlessness. Better understanding of the symptom is necessary for effective management. AIM: The aim was to describe the characteristics of episodic breathlessness in patients with advanced chronic obstructive pulmonary disease or lung cancer. DESIGN: This is a longitudinal cohort study. Outcomes were assessed monthly by up to 13 telephone interviews: peak severity (modified Borg scale: 0-10), duration, frequency, and timing of breathlessness episodes. Data from each episode were pooled and analyzed using descriptive statistics. Associations between outcomes were explored by correlation coefficients. SETTING/PARTICIPANTS: Patients with chronic obstructive pulmonary disease (Global Initiative for Chronic Obstructive Lung Disease classification stage III or IV) or primary lung cancer (any stage) were recruited in two inpatient units (internal medicine) and two outpatient clinics in Oldenburg, Germany. RESULTS: A total of 82 patients (50 chronic obstructive pulmonary disease, 32 lung cancer), mean age (standard deviation) 67 years (8 years) and 36% female, were included reporting on 592 breathlessness episodes (chronic obstructive pulmonary disease: 403, lung cancer: 189). Peak severity was perceived significantly higher in chronic obstructive pulmonary disease patients than in lung cancer patients (mean (standard deviation) Borg scale: 6.2 (2.1) vs 4.2 (1.9); p < 0.001). Episodes described by chronic obstructive pulmonary disease patients were longer than those described by lung cancer patients (median (range): 7 min (0-600) vs 5 min (0.3-120), p = 0.002)). Frequency was similar and most often daily in both groups. Severity and frequency of episodes were correlated in lung cancer patients (r = 0.324, p = 0.009). CONCLUSION: Most breathlessness episodes are short (minutes) and severe with significant differences between chronic obstructive pulmonary disease and lung cancer patients. Effective management strategies are warranted to improve symptom relief and coping.

Breathlessness, functional status, distress, and palliative care needs over time in patients with advanced chronic obstructive pulmonary disease or lung cancer: a cohort study.

CONTEXT: Breathlessness is a distressing symptom in advanced disease. Understanding its patterns, burden, and palliative care (PC) needs over time is important to improve patients' quality of life. OBJECTIVES: To describe and compare the courses of refractory breathlessness, functional status, distress, and PC needs in patients with advanced chronic obstructive pulmonary disease (COPD) or lung cancer (LC) over time. METHODS: This was a cohort study of patients with COPD (Stage III/IV) or LC. Data were assessed monthly with up to 12 telephone interviews, using the modified Borg Scale, Karnofsky Performance Status Scale, Distress Thermometer, and Palliative care Outcome Scale as outcomes measures. Descriptive analysis compared all outcomes between COPD and LC at baseline and over time (forward from study entry and backward from death). RESULTS: A total of 82 patients (50 COPD and 32 LC), mean (SD) age of 67.2 (7.8), and 36% female were included (8 COPD and 23 LC deceased). The patients with COPD perceived higher levels of breathlessness and distress at lower functional status steadily over time. The LC patients' breathlessness, distress, and PC needs increased, whereas functional status decreased toward death. The PC needs were similar between disease groups. Breathlessness was negatively correlated with functional status (COPD=mean r=-0.20, P=0.012; LC=mean r=-0.277, P=0.029) and positively correlated with PC needs in COPD patients (mean r=0.343, P<0.001). Death was significantly predicted by diagnosis (LC: hazard ratio=7.84, P<0.001) and functional status (10% decline: hazard ratio=1.52, P=0.001). CONCLUSION: The PC needs of patients with advanced COPD are comparable with LC patients, and breathlessness severity and distress are even higher. The care for COPD patients requires further improvement to address symptom burden and PC needs.

Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID).

INTRODUCTION: Evidence from a number of open-label, uncontrolled studies has suggested that rituximab may benefit patients with autoimmune diseases who are refractory to standard-of-care. The objective of this study was to evaluate the safety and clinical outcomes of rituximab in several standard-of-care-refractory autoimmune diseases (within rheumatology, nephrology, dermatology and neurology) other than rheumatoid arthritis or non-Hodgkin's lymphoma in a real-life clinical setting. METHODS: Patients who received rituximab having shown an inadequate response to standard-of-care had their safety and clinical outcomes data retrospectively analysed as part of the German Registry of Autoimmune Diseases. The main outcome measures were safety and clinical response, as judged at the discretion of the investigators. RESULTS: A total of 370 patients (299 patient-years) with various autoimmune diseases (23.0% with systemic lupus erythematosus, 15.7% antineutrophil cytoplasmic antibody-associated granulomatous vasculitides, 15.1% multiple sclerosis and 10.0% pemphigus) from 42 centres received a mean dose of 2,440 mg of rituximab over a median (range) of 194 (180 to 1,407) days. The overall rate of serious infections was 5.3 per 100 patient-years during rituximab therapy. Opportunistic infections were infrequent across the whole study population, and mostly occurred in patients with systemic lupus erythematosus. There were 11 deaths (3.0% of patients) after rituximab treatment (mean 11.6 months after first infusion, range 0.8 to 31.3 months), with most of the deaths caused by infections. Overall (n = 293), 13.3% of patients showed no response, 45.1% showed a partial response and 41.6% showed a complete response. Responses were also reflected by reduced use of glucocorticoids and various immunosuppressives during rituximab therapy and follow-up compared with before rituximab. Rituximab generally had a positive effect on patient well-being (physician's visual analogue scale; mean improvement from baseline of 12.1 mm). CONCLUSIONS: Data from this registry indicate that rituximab is a commonly employed, well-tolerated therapy with potential beneficial effects in standard of care-refractory autoimmune diseases, and support the results from other open-label, uncontrolled studies.

Palliative care in rheumatic diseases: a first approach.

Currently, the main goal in rheumatic research is to achieve remission, even in highly active stages of the disease. However, there is a lack of understanding of how to manage patients when some rheumatic diseases such as vasculitis, connective tissue disease, or rheumatoid arthritis develop fulminant, progressive, and complicated courses. There is a clear role for palliative care to enhance patients' quality of life, but hardly any data exist regarding the prevalence and management of symptoms, and the special needs of these patients and their relatives. Rheumatologists, and palliative and primary care physicians should become more aware of this patient group so as to offer them the care they need. Further research is necessary in this field.

[Pancytopenia, arthralgia and myeloneuropathy due to copper deficiency]. / Kupfermangel als seltene Ursache von Panzytopenie, Arthralgien und Gangstörungen.

BACKGROUND: Copper deficiency leads to hematologic disorders like pancytopenia. In addition, myeloneuropathy was described in a few cases reports. CASE REPORT: A 71-year-old woman was hospitalized because of increasing pancytopenia and ataxic gait, that resulted in a near-complete inability to walk without assistance. Additional symptoms included arthralgia, reduced appetite and weight loss. Laboratory studies revealed a proteinuria of 3,700 mg/day. Magnetic resonance imaging of the cervical and thoracic spine revealed a wedge-shaped signal intensity in the dorsal part as a sign of damage in this area. A copper deficiency was then identified as the likely underlying cause for the low blood cell counts and neurologic deficits. In this patient, the copper deficiency may have resulted from a disturbance in absorption due to a partial gastrectomy (modified Billroth I) 10 years ago and due to urinary copper loss in view of mesangioproliferative glomerulonephritis. A therapy with copper gluconate 3 x 3 mg/day was initiated. Within 2 weeks, blood cell counts normalized and appetite became normal again; just so, arthralgia disappeared. The neurologic symptoms persisted, even though the copper substitution continued for 6 months. CONCLUSION: Copper deficiency may be a differential diagnosis for hematologic abnormalities like pancytopenia, even if a disorder of intestinal resorption or a proteinuria occurs. Myeloneuropathy is a rare complication of this deficiency. Hemograms may become normal after treatment with oral copper gluconate, but at least in the case presented here, neurologic symptomes did not show any improvement.