Zeitschrift fur Rheumatologie最新文献

Background: Rheumatological care should be available for people with inflammatory rheumatic diseases in all regions of Germany. In this article the distance to rheumatological specialist care is mapped.

Methodology: Specialists in rheumatology listed in the German Federal Medical Register as of 31 December 2023 were assigned to postal code regions. The mean linear distance from a postal code region to the nearest region with a rheumatology practice was calculated. The mean distance from the places of residence of patients was calculated for the rheumatology centers participating in the German national database.

Results: Of the postal code regions 25% are > 20 km linear distance away from a postal code district with a rheumatological practice. Especially in Mecklenburg-Western Pomerania, Lower Saxony and Schleswig-Holstein there are larger areas with a low density of care. In the German national database, the median distance between the patient's place of residence and the rheumatological facility is between 10 km (Berlin) and 40 km (Tübingen).

Conclusion: In rural areas and for specialized rheumatology centers patients must travel long distances to reach a specialized rheumatological care.

Objective: This study aimed to evaluate and compare the diagnostic performance of immunoglobulin G4/Immunoglobulin G (IgG 4/IgG) and immunoglobulin G4 (IgG 4) alone in identifying immunoglobulin G4-related disease (IgG 4-RD).

Methods: A systematic review and meta-analysis were conducted using data from Medline, Embase, and the Cochrane Library from inception to November 2024. Two meta-analyses were performed to assess the diagnostic accuracies of IgG 4/IgG and IgG 4 in IgG 4-RD patients.

Results: Eight studies encompassing 754 IgG 4-RD patients and 9496 non-IgG 4-RD controls were included in the analysis. IgG 4/IgG demonstrated a sensitivity of 89% and a specificity of 91.6%, accurately detecting IgG 4-RD in 89% of cases and correctly identifying non-IgG 4-RD in 91.6% of cases. IgG 4 alone exhibited a higher sensitivity (94.9%) and a similar specificity (91%), indicating a slightly improved ability to identify IgG 4-RD cases. The positive likelihood ratio (PLR) and the negative likelihood ratio (NLR) for IgG 4/IgG were 7580 and 0.132, respectively, while IgG 4 alone had a PLR of 6403 and a lower NLR of 0.066, confirming the high diagnostic reliability. The diagnostic odds ratio (DOR) was 62.97 for IgG 4/IgG compared to 105.6 for IgG 4 alone, reflecting enhanced accuracy. The area under the curve (AUC) was 0.949 for IgG 4/IgG and 0.986 for IgG 4. The Q* index was 0.889 for IgG 4/IgG and 0.949 for IgG 4, further underscoring the diagnostic effectiveness of IgG 4 alone.

Conclusion: Both IgG 4/IgG and IgG 4 are highly accurate markers for diagnosing IgG 4-RD, with IgG 4 alone showing marginally higher sensitivity, DOR, and AUC. This suggests that IgG 4 alone may offer a slight advantage as a diagnostic marker for IgG 4-RD.

Background: Osteoporosis is a widespread disease defined by a reduction in bone mass and structure, thereby increasing the risk of fragility fractures. Treatment typically involves specific medications, which either inhibit bone resorption (antiresorptive) or stimulate bone formation (anabolic) and may potentially influence the healing of osteoporotic fractures. On the other hand, metabolic disorders, immune system dysfunctions or circulatory problems can impair fracture healing. Therefore, the targeted use of osteoporosis medications could be a strategy to promote the healing of impaired fractures.

Objective: The aim of this study is to provide a current overview of the effects of osteoporosis medications approved in Germany on fracture healing. The focus is on the potential influence of these medications in the context of osteoporosis treatment. Additionally, the current state of research is examined to explore to what extent the targeted use of these medications could improve fracture healing.

Material and methods: A literature search was conducted in the PubMed database using topic-specific keywords. Preclinical studies, clinical trials, review articles and meta-analyses were considered to present the current scientific knowledge with clinical relevance.

Results: Preclinical and clinical studies suggest that specific osteoporosis medications do not have a clinically relevant negative impact on the healing of fragility fractures. Osteoanabolic substances even tend to have a positive effect on fracture healing in both normal and impaired healing processes; however, the available studies are limited and none of the medications have been approved for this specific use.

Discussion: Osteoporosis medications with antiresorptive or osteoanabolic effects are primarily used to treat osteoporosis, especially after fragility fractures, to reduce the risk of further fractures. There is no clinically relevant impairment of fracture healing due to these medications. Further studies would be required to obtain approval for these medications specifically to improve fracture healing.

Background: Herbal preparations and phytotherapeutic substances are offered for symptoms and diseases of the rheumatic spectrum and are often intensively advertised in the lay press. The German Society for Rheumatology and Clinical Immunology (DGRh) Committee on Complementary Medicine and Nutrition reviewed the scientific literature on selected over the counter preparations and prescription phytotherapeutic substances and examined the possibilities for their use in rheumatology.

Methods: In an online meeting of the Committee on 8 February 2023 a list of herbal preparations that are frequently used in rheumatology (mostly as self-medication) was drawn up. Each member of the committee then carried out a literature search on one or two substances and summarized the results according to a defined matrix. Research was carried out on borage oil, stinging nettle preparations, cannabis preparations and preparations of dog rose, rosemary, saffron and willow bark. The data on the mixed preparation Phytodolor® (Bayer Vital GmbH, Germany) were also examined. The results were reviewed by a circulation procedure and approved in two further online meetings of the Committee. After review by the DGRh board, the recommendations were transferred to the professional organization's website.

Results: Even though there are reports of anti-inflammatory or immunological effects in vitro and/or in animal models for all the plant substances examined, the evidence for a clinically relevant benefit is sparse. None of the preparations investigated has a therapeutic efficacy that justifies its use in inflammatory joint diseases. Herbal preparations based on saffron and rosemary are generally not recommended. Borage oil from seeds can be taken in standardized form as part of a health-conscious diet but is not expected to have any significant anti-inflammatory effect. Rheumatologists need not advise against Phytodolor® or preparations based on stinging nettle, willow bark or dog rose, which are taken on the patient's initiative for degenerative joint diseases, if a sensible therapy concept is otherwise adhered to. There is insufficient evidence to prescribe medicinal cannabis for inflammatory rheumatic diseases for disease modification or symptomatic therapy. In individual cases, however, its use to reduce chronic pain, particularly neuropathic pain and sleep disorders and to reduce opiate consumption may be justified.

Conclusion: Even if the herbal preparations presented here must be considered in a differentiated manner for rheumatology practice, the value of phytotherapy for the discipline is low.

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children and adolescents. Currently, JIA is classified into seven categories according to the International League of Associations for Rheumatology (ILAR) criteria. Diagnosis is primarily clinical and involves excluding age-specific differential diagnoses, which can be particularly challenging in very young children. Early and effective treatment is crucial to minimize disease burden, chronic morbidity and reduced quality of life. Treatment strategies depend on the JIA category and comorbidities. The treatment should follow consensus treatment plans/strategies published by the German initiative Protocols for Classification, Monitoring and Therapy in Pediatric Rheumatology (ProKind) considering the treat-to-target strategy. Since a significant number of patients continue to have symptoms into adulthood, a well-structured transition from pediatric to adult rheumatology care is essential.

Pathological fractures under anti-osteoporotic and antirheumatic treatment are very rare events. Nevertheless, atypical femoral fractures occur during antiresorptive treatment with bisphosphonates or denosumab, the latter especially in patients previously treated with bisphosphonates. Treatment with teriparatide can be helpful. While glucocorticoids have a well-known influence on the development of osteoporosis and thus also fractures, the probably unproblematic use in the low-dose range has so far found little acceptance. Methotrexate-induced osteopathy is also a rare phenomenon but is now well accepted and known. There are several approved medications for the treatment of glucocorticoid-induced osteoporosis and for methotrexate-induced osteopathy, discontinuation of methotrexate is particularly essential.

Inflammatory rheumatic diseases, such as rheumatoid arthritis (RA), are characterized by local and systemic bone demineralization. Local demineralization is manifested in the periarticular region of the inflamed joints, particularly at the metacarpophalangeal and metatarsophalangeal joints. Local demineralization shows a significant correlation with inflammatory activity, whereas systemic osteoporosis, predominantly affecting the spine and hip, is typically associated with a prolonged disease duration, glucocorticoid treatment and immobilization. The receptor activator of the nuclear factor-kB ligand (RANKL)/osteoprotegerin (OPG) system and the Wnt signaling pathway play a pivotal role in regulating bone metabolism and are themselves negatively influenced by inflammatory cytokines such as tumor necrosis factor alpha (TNF-alpha) and interleukin (IL)-6. The diagnostics include both imaging procedures and procedures for bone mineral density measurement, employing techniques such as high-resolution peripheral quantitative computed tomography (HR-pQCT) and dual energy X‑ray absorptiometry (DXA). These are used for quantification of the bone mineral density. The objective of therapeutic approaches is to reduce disease activity and modulate signaling pathways in order to slow down demineralization and reduce the risk of fractures.

Pregnancy and lactation-associated osteoporosis (PLO) is a rare but serious condition. Multiple fractures often occur, mostly in the form of vertebral fractures, the mother is severely restricted and caring for the infant is barely possible without assistance. The fractures causing the complaints usually occur in the last trimester of the first pregnancy or in the first weeks of lactation. Magnetic resonance imaging (MRI) can be used to detect vertebral fractures and also edematous vertebrae. Bone densitometry is helpful for the diagnostics and assessment of progression. It is extremely important to distinguish PLO from other secondary forms of osteoporosis that can also be manifested during pregnancy and lactation. The mother is advised to stop breastfeeding immediately in order to interrupt calcium mobilization from bone and to achieve a normalization of hormone levels. Calcium and vitamin D should be supplemented and adequate pain treatment and physiotherapy should be initiated. The quality of data is poor due to the rarity of the disease, all available anti-osteoporotic drugs have been used in case reports but overall, in the last decade off-label treatment with teriparatide has been proven to be helpful and safe.

Metabolic bone diseases cause bone and joint pain and are manifested as rheumatism. Typical for the rare genetic disease hypophosphatasia is a reduced activity of alkaline phosphatase (AP), where the variable residual activity causes the heterogeneous symptoms (e.g., arthralgia, myalgia and fractures). It is indicated by repeatedly low AP measurements. The diagnosis requires a meticulous medical history and laboratory-based clarification in order to rule out other differential diagnoses. Although supportive measures form the basis of treatment, costly enzyme replacement therapy is a possible treatment option for severe forms. Multidisciplinary care under the direction of a rheumatologist experienced in osteology or an osteologist is crucial in order to provide adequate care to affected patients. Phosphate loss syndromes due to overactivity of fibroblast growth factor 23 (FGF-23) lead to deformities of the lower extremities and short stature (in congenital disorders), bone and muscle pain, muscular weakness and pathological fractures, depending on the time of occurrence during life. In genetic forms of the disease (especially X‑linked hypophosphatemia), supplementation with calcitriol and phosphates and, if necessary, complex corrective surgery in adolescence are traditional treatment methods, which are increasingly being replaced by treatment with antibodies against FGF-23. The acquired variant is a paraneoplastic phenomenon from small mostly benign mesenchymal tumors, which clinically shows a relatively acute course with severe bone pain, pathological fractures and muscle weakness in previously healthy patients and can ideally be cured by resection of the tumor. The disease can be suspected by significantly reduced serum phosphate levels and narrowed down with further laboratory diagnostics. In our opinion, the measurement of calcium, phosphate and alkaline phosphatase should be part of the primary laboratory diagnostics performed by rheumatologists and the follow-up of pathological findings is indicated.