Zeitschrift fur Rheumatologie最新文献

Fever can be due to infectious or noninfectious causes and results from the body's natural response to exogenous or endogenous pyrogens. Laboratory tests including complete blood count, differential blood count, C‑reactive protein, erythrocyte sedimentation rate and procalcitonin do not have sufficient sensitivity and specificity to definitively detect or rule out an infectious (bacterial, viral, parasitic) cause of fever. Blood cultures should be carried out when bacteremic or septic illnesses are suspected. Fever is not always present in infections and can be absent, especially in older and immunocompromised patients. If fever is suspected, core temperatures should be taken, e.g., rectally, orally or invasively. Depending on the clinical situation, infectious causes must be excluded as the most likely cause of an acutely occurring fever. The investigation of long-standing fever (fever of unknown origin, FUO) can be complex and some infectious diseases should first be ruled out, whereby a syndromic classification often helps to clarify the cause of the fever.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that is characterized by hyperferritinemia, cytopenia, disseminated intravascular coagulopathy and functional disorders of the liver and the central nervous system. The term macrophage activation syndrome is predominantly used for secondary HLH in the context of autoimmune diseases (e.g., systemic juvenile idiopathic arthritis). In addition, malignancies and genetic inborn errors of immunity can predispose to the development of HLH. Infections (e.g., Epstein-Barr virus) in turn represent possible triggers of an acute episode. Due to the unspecific manifestation of the disease, a systematic evaluation of the organ systems is recommended in the clinical and laboratory analytical clarification of hyperinflammatory syndromes. In general, the treatment should be carried out by a multidisciplinary team with expertise in rheumatology, hematological oncology, infectious diseases and intensive care medicine. The primary treatment of HLH usually consists of glucocorticoids and in cases of a rapid deterioration of the condition anakinra (interleukin 1 block) and intravenous immunoglobulins can be employed. Treatment of the underlying disease should be consequently carried out in parallel, together with antimicrobial treatment.

Lysosomal storage diseases are a group of rare hereditary metabolic diseases. Due to a deficiency of lysosomal enzymes, complex substrates accumulate in the lysosomes of various organs. Depending on the affected enzyme, this results in clinically variable and chronic progressive multiorgan diseases. Diagnosis is often delayed. As clinical symptoms include the musculoskeletal system, an awareness of lysosomal storage diseases is of relevance to (pediatric) rheumatologists. This article is focused on Mucopolysaccharidosis type I‑S, Mucolipidosis type III, Gaucher disease and Fabry disease. When suspecting a lysosomal storage disease, enzyme activity should be determined in dried blood spots or leukocytes. For some diseases, specific biomarkers can additionally be analyzed. Diagnosis should be confirmed by genetic testing. As causal treatment options are available for three of the presented diseases, a timely diagnosis is very important.

Background/objectives: Current data on the care of patients with vasculitis in Germany are scarce. Patient-reported outcome (PRO) questionnaires can capture aspects of the disease that escape conventional scores for disease activity, remission, and damage. For this reason, the Association of Rheumatological Acute Care Clinics (VRA) initiated a data analysis as part of the KOBRA quality project, the results of which are presented here.

Patients and methods: Patients with vasculitis of vessels of any size or with polymyalgia rheumatica were included. The prospective survey included data on demographics, disease, pain, treatment, follow-up and satisfaction at the time of inpatient admission, discharge and follow-up after 2.5 months. All patients completed the AAV-PRO and EQ-5D-3L questionnaires on admission and follow-up.

Results: In this study 420 patients were recruited and follow-up data were available from 302. On average, improvements were documented in all 5 dimensions of the EQ-5D, with the strongest effects in self-care and coping with activities of daily living. In the AAV-PRO, highly significant differences were seen in the domains systemic symptoms and physical functioning. Satisfaction with medical and nursing treatment was very high and did not correlate with pain level or with the AAV-PRO measures.

Discussion: Under zreatment patient-reported outcomes improve at least partially in vasculitis patients. Satisfaction with medical treatment quality is independent of these outcomes.

Autoinflammatory diseases are characterized by inflammatory manifestations in various organ systems, whereby recurrent febrile episodes, musculoskeletal complaints, gastrointestinal and cutaneous symptoms frequently occur accompanied by serological signs of inflammation. Autoinflammatory diseases include rare monogenic entities and multifactorial or polygenic diseases, which can manifest as a variety of symptoms in the course of time. Examples of monogenic autoinflammatory diseases are familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) and the recently described VEXAS (vacuoles, E1 enzyme, X‑linked, autoinflammatory and somatic) syndrome. For non-monogenically determined autoinflammatory diseases, the most important representatives in adulthood are adult-onset Still's disease (AOSD) and the Schnitzler syndrome, in which a polygenic susceptibility and epigenetic factors are more likely to play a role.

Background: The adaptation of structures and processes in treatment procedures can contribute to increasing patient satisfaction and is the focus of patient-oriented quality assurance.

Objective: To identify patient satisfaction as well as needs, expectations and preferences with respect to care and, based on this, to formulate recommendations for action to optimize the quality of care at a large tertiary rheumatology center.

Material and methods: As part of a qualitative research approach, semi-structured patient interviews and a focus group interview consisting of physicians in rheumatology training in outpatient specialist care were conducted. The quality dimensions of Donabedian were recorded. The data material was evaluated and analyzed using the content-structuring qualitative content analysis according to Kuckartz with the MAXQDA evaluation software.

Results: Using 12 patient interviews and a focus group of 3 future rheumatologists, recommendations for action to optimize the quality of care were derived on the basis of the structural, process and outcome quality. There was a need for optimization in the areas of personnel management, internal practice processes, practice equipment and treatment processes in the outpatient clinic.

Conclusion: The results from the patient interviews and the focus group revealed the aspects in need of optimization. The methodology and results of this study can serve as a reference point for analyses of other rheumatology clinics in order to improve the quality of care within the framework of patient-oriented quality management and continuous further development.