[Ultrasound examination of nerves of the upper extremities]. / Nervensonographie der oberen Extremitäten.

The ultrasound examination of peripheral nerves has been further developed in recent years and is recognized as an independent discipline by the German Society of Ultrasound in Medicine (DEGUM). A systematic ultrasound examination of the musculoskeletal system is not limited to the joints, muscles and bones but should also include the examination of nerves and blood vessels. Therefore, in the practice of ultrasound examination every rheumatologist should have at least a basic knowledge of the ultrasound examination of the peripheral nerves. In this article the authors present a landmark-based concept in which the three large nerves of the upper extremities can be completely visualized from proximal to distal and evaluated.

[Rehabilitation of orphan diseases in adulthood: osteogenesis imperfecta]. / Rehabilitation seltener Erkrankungen im Erwachsenenalter: Osteogenesis imperfecta.

Osteogenesis imperfecta (brittle bone disease) is an orphan disease caused by a genetic mutation in collagen metabolism. Bone fractures are the most common symptoms; however, the clinical manifestation can vary widely. Additional features can include blue sclera, dwarfism, bone deformities, muscular weakness, scoliosis, hearing loss and hypermobility of joints. Most patients show a reduction of skeletal function. This leads to an increased risk of being unable to continue their former work and to participate in social life. A comprehensive treatment includes drug therapy, surgery and rehabilitation. This article gives an overview of the current status of rehabilitation in adult patients with osteogenesis imperfecta.

[Osteomalacia-Clinical aspects, diagnostics and treatment]. / Osteomalazie ­ Klinik, Diagnostik und Therapie.

Osteomalacia is a bone disease caused by impaired skeletal mineralization. Vitamin D dependent types have to be distinguished from hypophosphatemic forms. Typical signs and symptoms include diffuse bone pain, muscle weakness and fragility fractures. The fracture pattern in osteomalacia is typically different from that of osteoporosis. Fragility fractures of the pelvis, sacrum, distal parts of the foot, proximal tibia and ribs are indicators for osteomalacia, whereas femoral neck and vertebral fractures (wedged vertebra, fish vertebra, vertebra plana and cover plate impression fractures) are typical for osteoporosis. Unspecific clinical features may be the reason for a delayed diagnosis. The correct classification of the complaint is dependent on the knowledge of the pathophysiology of osteomalacia and performance of additional bone-specific examinations. Determination of specific laboratory parameters should follow a rational algorithm, supplemented by imaging methods and a bone biopsy.

[Pregnancy and lactation-associated osteoporosis]. / Schwangerschaftsassoziierte Osteoporose.

Pregnancy and lactation-associated osteoporosis (PLO) is a rare form of osteoporosis, which occurs in the last trimester or postpartum. So far 100 cases have been published. The leading symptoms are severe low back pain or less frequently hip pain. Many patients develop postpartum depression due to inability to care for the baby and vertebral fractures. The therapeutic decision has to be made individually but teriparatid and bisphosphonates seem to be the best option. We report the clinical course (16 years) of a 37-year-old patient with PLO, who suffered 6 vertebral fractures. There were severe physical limitations and mental problems caused by the disease. The patient was treated by multimodal therapy including physiotherapy and psychotherapy and bisphosphonates were given. The time between the onset of symptoms and diagnosis was 5 months. No further fractures occurred in the following 16 years. The physical and mental condition significantly improved.

[Severe therapy refractive osteoporosis : A rare differential diagnosis]. / Schwere therapierefraktäre Osteoporose : Eine seltene Differenzialdiagnose.

Systemic mastocytosis is defined as a clonal increase of mast cells. We report on four patients with severe osteoporosis and histologically confirmed systemic mastocytosis. In spite of antiresorptive therapy the patients developed further vertebral fractures and suffered from ostealgia. Systemic mastocytosis is an important differential diagnosis in patients with therapy refractive and unexplained osteoporosis. Skin involvement (urticaria pigmentosa) is a rare occurrence and in most cases an isolated involvement of bone marrow is present. Determination of serum tryptase can provide indications for systemic mastocytosis but the diagnosis is only confirmed by bone marrow biopsy. There is a high risk of vertebral fractures and patients should be treated in specialized centers. Zoledronic acid can be a therapeutic option for indolent osteoporosis associated with systemic mastocytosis.

[Brain stem infarction, temporal headache, and elevated inflammatory parameters in a 74-year-old man]. / Hirnstamminfarkt, temporaler Kopfschmerz und erhöhte Entzündungsparameter bei einem 74-jährigen Patienten.

We report the case of a 74 year old man with a brain stem infarction, temporal headache and elevated inflammatory parameters. Giant cell arteritis with involvement of the temporal and vertebral arteries was proven by histology, duplex sonography and MRI. Although intensive immunosuppressive therapy was started, the patient developed two brain infarcts within 6 months. Initially, C­reactive protein and erythrocyte sedimentation rate were significantly elevated, but normalized over time. Involvement of the vertebral artery in giant cell arteritis is thought to be rare; steroid refractory courses are very rare. Brain stem infarction might be the consequence.

[A 58-year-old patient with temporal headache, jaw claudication and B symptoms]. / Ein 58-jähriger Patient mit temporalen Kopfschmerzen, Kauclaudicatio und B-Symptomatik.

CASE REPORT: We report the case of a patient with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with involvement of the temporal artery, presenting with clinical manifestations of giant cell arteritis and temporal arteritis, such as temporal headache, jaw claudication, weight loss, night sweats and increased inflammatory parameters. The ultrasound scan showed a typical halo sign of the temporal artery. DIAGNOSTICS: In the case of further atypical organ symptoms, e.g. hematuria and proteinuria, detailed differential diagnostic investigations are essential to clarify the situation. Histological findings from the affected organs play a decisive role. CONCLUSION: An involvement of the temporal artery due to ANCA-associated vasculitis is extremely rare and may mimic giant cell arteritis. The exact diagnosis of ANCA-associated vasculitis is, however, important because this leads to a different approach concerning therapy and prognosis.

[A 48-year-old patient with oligoarthritis of the knees, having excluded spondyloarthritis and rheumathoid arthritis]. / 48-jähriger Patient mit Schmerzen und Schwellungen beider Kniegelenke bei Ausschluss einer Spondyloarthritis und einer rheumatoiden Arthritis.

We report the case of a patient with amyloid light-chain (AL) amyloidosis, presenting for more than 1.5 years with oligoarthritis as the only clinical symptom of the underlying disease. Developing further organ symptoms (heart and gastrointestinal tract) led to the definitive diagnosis. Joint involvement due to AL amyloidosis is extremely rare and may mimic rheumatoid arthritis. Arthritis might be the first symptom of the AL amyloidosis. The combination of arthritis and other organ disorders (e.g., proteinuria, heart failure, or diarrhea) should initiate further diagnostic efforts considering AL amyloidosis as a differential diagnosis. The diagnosis will be confirmed by the typical histology of synovia or involved organs including immunohistochemistry for typing amyloid and performing immunoelectrophoresis.

[Care of the dying in the hospital: initial experience with the Liverpool Care Pathway (LCP) in Germany]. / Sterbebegleitung im Krankenhaus--erste Erfahrungen mit dem "Liverpool Care Pathway" (LCP) in Deutschland.

BACKGROUND AND OBJECTIVE: In the last few years public interest in the care of severely ill and dying patients has been growing. The aim of palliative medicine is to improve the care of the dying. However, this is still not achieved in many general hospitals. The Liverpool Care Pathway (LCP) for the care of the dying intends to change this situation. The aim of this study was to explore the views of professionals using the LCP as a framework for ensuring good care of the dying. METHODS: A qualitative study was conducted with an interdisciplinary focus group of ten professionals (nurses, physicians, spiritual adviser, social worker, physiotherapist and art therapist) to explore their views and experience after implementation of the LCP in a palliative care unit (PCU). The recorded discussion between them was transcribed verbatim and analysed using content analysis by three independent reviewers. RESULTS: Seven nurses and three physicians with an average work experience of 16 years each took part in the focus group. Based on the experience of 24 patients, the LCP was evaluated as very positive by all participants. In particular, three aspects were emphasized as having high relevance for a good quality of care: improvement of self-confidence, better control of symptoms, and enhancement of the communication between professionals and with patients and their relatives. However, some weaknesses were also mentioned, e.g. inadequate effort of documenting the beginning of implementing the scheme. CONCLUSION: The LCP was well received by professionals after the initial implementation of the LCP in a German PCU. The LCP was judged as an appropriate and helpful framework in the care of the dying.

[Fulminant course of a Takayasu's arteritis and rare mesenteric arterial maninfestion]. / Fulminanter Verlauf einer Takayasu-Arteriitis und seltener mesenterialer Gefässbefall.

Takayasu's arteritis is a systemic disease, presenting as chronic inflammation of the main arteries. It usually affects the aorta and its large branches. General symptoms often include fatigue, subfebrile temperatures and weight loss. Complaints due to perfusion disorders are, for example, muscle pain, dizziness or claudication. Takayasu's arteritis is a rare disease which generally occurs in female patients under 40 years of age. We report on a patient with primary involvement of the mesenteric arteries. The disease process was fulminant and refractory to all therapeutic strategies. According to our knowledge this is the first case report from Germany of Takayasu's arteritis with mesenteric infarction.

[Rheumatoid arthritis at the cervical spine -- an underestimated problem]. / Die rheumatoide Arthritis an der Halswirbelsäule: ein unterschätztes Problem.

BACKGROUND AND OBJECTIVE: The involvement of the cervical spine in rheumatoid arthritis can be essential regarding prognosis and mortality. The cervical myelopathy due to pannus formation and/or subluxation can be fatal. Aim of this study was to demonstrate the possible changes seen by MRI, and to establish a risk-profile for the individual patient. PATIENTS AND METHOD: Within a period of 24 months 214 patients with active RA were included. Clinical and laboratory data were obtained and plain radiographs of the cervical spine were taken. In patients with pathological findings on X-ray an MRI was performed (36 patients). RESULTS: Within the group of 214 patients 36 were identified to get an cervical spine MRI. In all cases the MRI showed significant changes: in 7 (19.5 %) pannus surrounded the dens, with additional erosions in one patient (2.7 %). In 25 (69.5 %) atlanto-axial-subluxation was present, 7 (19.5 %) showed a spondylodiscitis below C2. In 10 (27.8 %) a cervical myelopathy due to pannus or subluxation was present. There was no correlation of the MRI-results with symptoms and findings by examination. The patients with cervical spine disease were in all stages of RA. The majority was rheumatoid-factor positive. 5 out of 10 patients with cervical myelopathy showed neurological deficits: 3 patients died in consequence of neural compression, 2 patients underwent surgery successfully. CONCLUSION: The early detection of a cervical spine involvement in RA is essential to avoid possibly fatal complications. The only reliable method to achieve this goal has to include radiographic diagnostic including MRI of the cervical spine. Only this approach can answer the question of the right time-point for surgery. In daily clinical practice the cervical-spine involvement in RA is still underestimated.

Loss of collagen type IV in rheumatoid synovia and cytokine effect on the collagen type-IV gene expression in fibroblast-like synoviocytes from rheumatoid arthritis.

Collagen type IV is a structural matrix protein which contributes to the structural organization of the synovia. In order to characterize the distribution of this protein in synovia with chronic synovitis, collagen type IV was detected by immunochemistry in normal synovia and in synovia from patients with osteoarthritis (OA) and rheumatoid arthritis (RA). A decrease of collagen type IV was observed in synovial layers of rheumatoid synovia, which statistically correlated with the grade of inflammation and with the thickness of the synovial layer. In vitro, we found no differences in the gene expression of collagen type IV in cultures of fibroblast-like synoviocytes (FLS) derived from OA and RA using a reverse-transcriptase polymerase chain reaction. Nevertheless, we observed a downregulating effect of tumor necrosis factor-alpha and interleukin (IL)-1beta on the gene expression of collagen type IV only in FLS isolated from patients with RA. The effect of IL-1beta was dose dependent. In summary, we observed an inflammation-associated decrease of collagen type IV in the synovial layer of rheumatoid synovia. Inflammatory cytokines may play a role in regulating the synthesis of collagen type IV in the rheumatoid process in vivo.

Seltene Manifestation einer Grossgefassvaskulitis bei|| systemischem Lupus erythematodes.

Case-reports from two female patients with an occlusion||| of the right external iliac artery and femoral artery are presented due to a||| large-vessel vasculitis. Both patients suffered from systemic lupus||| erythematosus This rare manifestation occurred within the first few years of||| the disease and was important for prognosis and further treatment. Other||| manifestations of the disease were general symptoms and polyarthritis. In one||| case the vasculitis was confirmed by histology. A fibrous thickening of the||| intima and a vasculitis of small vessels within the adventitia were the||| prominent feature. This observation supports the idea of small vessel||| vasculitis as the characteristic manifestation in lupus||| erythematosus.

[Relevance of a standardized staging study in patients with systemic vasculitis]. / Die Relevanz einer standardisierten Staging-Untersuchung bei Patienten mit systemischer Vaskulitis.

Primary systemic vasculitities play an important role in the differential diagnosis of systemic diseases. They can be classified according to newly developed classification criteria. In the past, systemic vasculitities were more diagnosed in the general phase of the disease. In the meantime limited forms can be diagnosed more easily. Prior to immunosuppressive therapy, a standardized staging-procedure is essential to define the extent of the disease. Therefore a program was established using methods that are easy to apply in all patients. In this setting the usefulness of this approach was investigated. Apart from the laboratory tests including antibody testing, the chest x-ray, consultation of the ENT specialist, ultrasound of the abdomen, and ECG were also very useful. The eye and neurological investigations, and the MRI of the head were of minor significance, but also important.

[Rare manifestation of large vessel vasculitis in systemic lupus erythematosus]. / Seltene Manifestation einer Grossgefässvaskulitis bei systemischem Lupus erythematodes.

Case-reports from two female patients with an occlusion of the right external iliac artery and femoral artery are presented due to a large-vessel vasculitis. Both patients suffered from systemic lupus erythematosus This rare manifestation occurred within the first few years of the disease and was important for prognosis and further treatment. Other manifestations of the disease were general symptoms and polyarthritis. In one case the vasculitis was confirmed by histology. A fibrous thickening of the intima and a vasculitis of small vessels within the adventitia were the prominent feature. This observation supports the idea of small vessel vasculitis as the characteristic manifestation in lupus erythematosus.

[Immunosuppressive therapy in autoimmune diseases]. / Immunsuppressive Therapie von Autoimmunerkrankungen.

Autoimmune diseases play an increasing role in daily practise. Advanced understanding of the pathogenesis and pathophysiology enables an outcome orientated therapy leading to a far better prognosis. Immunosuppressive drugs are essential for the treatment. The therapeutical concepts are adopted to the individual character and stage of the disease. In the meantime, therapeutical regimes combine certain drugs. Although it is not possible to cure these autoimmune diseases, a remission can be achieved in most cases. Further developments will concentrate on more selective strategies and the potentials of gene therapy.

[Molecular mechanisms of cell-cell and cell-matrix interactions in the pathophysiology of rheumatoid arthritis]. / Molekulare Mechanismen der Zell-Zell- und Zell-Matrix-Interaktionen in der Pathophysiologie der Rheumatoiden Arthritis.

The original structure of the synovial membrane is completely destroyed in the rheumatoid synovium and is characterized by mononuclear cell infiltration, synoviocyte proliferation, neo-vascularization, and deposition of extracellular matrix proteins. Adhesion molecules play an important role in the development of these pathologic changes. In this review we discuss the role of the adhesion receptors of the selectin, integrin and immunoglobulin families and of the CD44 molecule in the cell-cell and cell-matrix interaction in the pathogenesis of the inflammatory changes in rheumatoid arthritis.

Loss of laminin and of the laminin receptor integrin subunit alpha 6 in situ correlates with cytokine induced down regulation of alpha 6 on fibroblast-like synoviocytes from rheumatoid arthritis.

OBJECTIVE: To investigate in situ the expression of the integrin receptor subunits alpha 6 and beta 1 and the distribution of the ligand laminin in the synovia from osteoarthritis (OA) and rheumatoid arthritis (RA) patients and to study the effect of cytokines and antirheumatic drugs on the expression of the alpha 6 and beta 1 integrin subunits on long term cultures of fibroblast-like synoviocytes (FBS) derived from OA and RA. METHODS: The expression of the alpha 6 and beta 1 integrin subunits and the distribution of laminin were examined immunohistochemically in normal synovia and in synovia from patients with OA and RA. The effect of proinflammatory cytokines (IL1 beta and TNF alpha), and of antirheumatic drugs (salicylic acid, dexamethasone, and methotrexate) on the alpha 6 and beta 1 expression of cultured normal FBS and FBS from patients with OA and RA was determined by flow cytometry. RESULTS: In normal synovia and in OA synovia samples with a low grade of inflammation, synovial lining cells (SLC) showed a parallel expression and distribution of alpha 6 and laminin. In synovia samples of OA with a higher grade of inflammation and in the majority of RA synovia samples laminin was pericellularly distributed in a low number of SLC, whereas alpha 6 was expressed on the surface of a high number of SLC. In RA synovia samples with severe inflammatory changes the gradual loss of laminin generally corresponded to a decrease of the alpha 6 integrin subunit. beta 1 was always strongly expressed in all synovia samples detected. Proinflammatory cytokines up regulated the expression of alpha 6 and beta 1 on OA-FBS, whereas these effectors decreases alpha 6 and beta 1 on RA-FBS. In contrast, antirheumatic drugs, in particular methotrexate and dexamethasone, reduced the expression of alpha 6 and beta 1 on OA-FBS, whereas the same treatment on RA-FBS stimulated the expression of these integrin subunits. CONCLUSION: The gradual loss of laminin in chronic synovitis may contribute to the altered expression of alpha 6 in SLC. IL1 beta and TNF alpha down regulated the expression of the alpha 6 and beta 1 integrin subunits on long term cultures of FBS derived from RA. Therefore, these cytokines may be among the effectors regulating the expression of the alpha 6 integrin subunit in SLC in vivo. As antirheumatic drugs increase the expression of alpha 6 on RA-FBS, the presence of the laminin receptor may confer a protective effect on the synovia in vivo.

Methotrexate in patients with moderate systemic lupus erythematosus (exclusion of renal and central nervous system disease).

OBJECTIVES: Methotrexate (MTX) has been used in several autoimmune diseases. Apart from its use in rheumatoid arthritis, MTX has been assessed in small studies in patients with vasculitis, uveitis, and inflammatory bowel disease. The aim of this study was to evaluate the efficacy of MTX in a particular group of patients with systemic lupus erythematosus (SLE). PATIENTS: In an open prospective study 22 patients fulfilling the ACR criteria for SLE were included. Patients had one or more of the following manifestations; active non-destructive polyarthritis, dermatitis, vasculitis of the skin, pleuritis. All patients had been treated with corticosteroids for at least six months without achieving remission. Sixteen patients were taking antimalarial drugs in addition to corticosteroids, which were stopped at the beginning of the trial. Patients with renal and central nervous involvement were excluded from the study. All patients received MTX orally at a dose of 15 mg/week over six months. Corticosteroids were continued. As additional medication only indomethacin up to 100 mg/day was permitted if used before the start of the study. The outcome was evaluated using the SLE disease activity index (SLEDAI). RESULTS: Disease activity was evaluated after six months of MTX treatment. All patients completed the study period. The SLEDAI decreased significantly from mean (SD) 12.2 (3.99) to 4 (3.75) (p = 0.001). The prednisolone dose was reduced from a mean (SD) of 17.4 (12.8) at the beginning to 8.8 (5.36) mg/day at the end point of the study (p = 0.01). MTX was well tolerated. Four patients complained of general malaise. Two patients had transient increases in liver enzymes. In no case did MTX have to be stopped. CONCLUSIONS: In an open prospective study methotrexate was used in SLE patients with particular clinical characteristics. MTX was shown to be effective in reducing disease activity and sparing the dose of corticosteroids. Further controlled studies are necessary.