Hemophagocytic lymphohistiocytosis and macrophage activation syndrome : A multidisciplinary challenge
Z Rheumatol. 2024 Jan 24. doi: 10.1007/s00393-023-01472-w. Online ahead of print.ABSTRACTHemophagocytic 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....
Source: Zeitschrift fur Rheumatologie - January 24, 2024 Category: Rheumatology Authors: Nikolas Ruffer Ricardo Kosch Katja Weisel Ina K ötter Martin Krusche Source Type: research

Screening for rheumatoid arthritis-associated interstitial lung disease-a  Delphi-based consensus statement
Z Rheumatol. 2024 Jan 19. doi: 10.1007/s00393-023-01464-w. Online ahead of print.ABSTRACTOBJECTIVE: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is a major driver of premature mortality in patients with rheumatoid arthritis (RA). Detection of RA-ILD is crucial but requires awareness among the treating physicians. To date, however, there is no international recommendation concerning screening for ILD in RA patients.METHODS: After a systematic literature review, the modified Delphi technique in combination with the nominal group technique was used to provide a Delphi consensus statement elaborated by an...
Source: Zeitschrift fur Rheumatologie - January 19, 2024 Category: Rheumatology Authors: Klaus Hackner Lisa H ütter Holger Flick Michael Grohs Kastriot Kastrati Hans Kiener David Lang Birgit Mosheimer-Feistritzer Helmut Prosch Eva Rath Otmar Schindler Florentine Moazedi-F ürst Source Type: research

Sarilumab for polymyalgia rheumatica
Z Rheumatol. 2024 Jan 19. doi: 10.1007/s00393-024-01474-2. Online ahead of print.NO ABSTRACTPMID:38240818 | DOI:10.1007/s00393-024-01474-2 (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - January 19, 2024 Category: Rheumatology Authors: Wolfgang A Schmidt Source Type: research

Phalangeal microgeodic syndrome: case report of a  young woman treated with pentoxifylline
We report the case of a young woman presenting with joint pain in her feet, along with toe discoloration and redness, where a diagnosis of PMS was established based on magnetic resonance imaging findings and exclusion of other differential diagnostic entities. Pharmacological treatment was deemed necessary for symptomatic relief, but a trial of calcium channel blocker (CCB) was not tolerated by the patient. The patient was then started on pentoxifylline, with significant clinical improvement.PMID:38175270 | DOI:10.1007/s00393-023-01470-y (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - January 4, 2024 Category: Rheumatology Authors: Tiago Borges Miguel Castro Source Type: research

Phalangeal microgeodic syndrome: case report of a  young woman treated with pentoxifylline
We report the case of a young woman presenting with joint pain in her feet, along with toe discoloration and redness, where a diagnosis of PMS was established based on magnetic resonance imaging findings and exclusion of other differential diagnostic entities. Pharmacological treatment was deemed necessary for symptomatic relief, but a trial of calcium channel blocker (CCB) was not tolerated by the patient. The patient was then started on pentoxifylline, with significant clinical improvement.PMID:38175270 | DOI:10.1007/s00393-023-01470-y (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - January 4, 2024 Category: Rheumatology Authors: Tiago Borges Miguel Castro Source Type: research

Phalangeal microgeodic syndrome: case report of a  young woman treated with pentoxifylline
We report the case of a young woman presenting with joint pain in her feet, along with toe discoloration and redness, where a diagnosis of PMS was established based on magnetic resonance imaging findings and exclusion of other differential diagnostic entities. Pharmacological treatment was deemed necessary for symptomatic relief, but a trial of calcium channel blocker (CCB) was not tolerated by the patient. The patient was then started on pentoxifylline, with significant clinical improvement.PMID:38175270 | DOI:10.1007/s00393-023-01470-y (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - January 4, 2024 Category: Rheumatology Authors: Tiago Borges Miguel Castro Source Type: research

Phalangeal microgeodic syndrome: case report of a  young woman treated with pentoxifylline
We report the case of a young woman presenting with joint pain in her feet, along with toe discoloration and redness, where a diagnosis of PMS was established based on magnetic resonance imaging findings and exclusion of other differential diagnostic entities. Pharmacological treatment was deemed necessary for symptomatic relief, but a trial of calcium channel blocker (CCB) was not tolerated by the patient. The patient was then started on pentoxifylline, with significant clinical improvement.PMID:38175270 | DOI:10.1007/s00393-023-01470-y (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - January 4, 2024 Category: Rheumatology Authors: Tiago Borges Miguel Castro Source Type: research

Phalangeal microgeodic syndrome: case report of a  young woman treated with pentoxifylline
We report the case of a young woman presenting with joint pain in her feet, along with toe discoloration and redness, where a diagnosis of PMS was established based on magnetic resonance imaging findings and exclusion of other differential diagnostic entities. Pharmacological treatment was deemed necessary for symptomatic relief, but a trial of calcium channel blocker (CCB) was not tolerated by the patient. The patient was then started on pentoxifylline, with significant clinical improvement.PMID:38175270 | DOI:10.1007/s00393-023-01470-y (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - January 4, 2024 Category: Rheumatology Authors: Tiago Borges Miguel Castro Source Type: research

Advances in the diagnostics and treatment of juvenile dermatomyositis
Z Rheumatol. 2023 Dec 29. doi: 10.1007/s00393-023-01454-y. Online ahead of print.ABSTRACTJuvenile dermatomyositis (JDM) is by far the most frequent inflammatory myopathy in childhood and adolescence. It is clinically characterized by inflammatory changes of the skin and muscles but as a multisystemic disease can also affect the skeletal system, the gastrointestinal tract, lungs and heart. Intrinsic (multigenetic risk) and extrinsic factors (triggers) are involved in the pathogenesis resulting in endothelial damage, involvement of fascies, activation of the interferon system and autoimmune reactions including formation of m...
Source: Zeitschrift fur Rheumatologie - December 29, 2023 Category: Rheumatology Authors: Ingrid Georgi Mathias Georgi Johannes-Peter Haas Source Type: research

Protocols in pediatric rheumatology (PROKIND): treat-to-target in polyarticular juvenile idiopathic arthritis
CONCLUSION: Using a treat-to-target approach a dramatic improvement in disease activity, functional capacity and quality of life in polyarticular JIA could be achieved. Even after 12 months an inactive disease was achieved in the majority of cases.PMID:38157052 | DOI:10.1007/s00393-023-01452-0 (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - December 29, 2023 Category: Rheumatology Authors: G Horneff K Minden D Foell J Klotsche K Tenbrock PROKIND-Arbeitsgruppe Source Type: research

Safety and efficacy of telitacicept in refractory systemic lupus erythematosus patients who failed treatment with belimumab : A  case series
CONCLUSION: This is the first case series in SLE patients who accepted telitacicept treatment after failed treatment with belimumab. Our case series and review of the literature show that telitacicept combined with the original standard treatment may significantly improve disease activity while reducing prednisone use. No major safety issues were seen in this group of patients. Telitacicept may be a promising drug for the treatment of refractory lupus hepatitis.PMID:38157053 | DOI:10.1007/s00393-023-01461-z (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - December 29, 2023 Category: Rheumatology Authors: Qiuyu Fan Huiqin Yang Ya Liu Source Type: research

Advances in the diagnostics and treatment of juvenile dermatomyositis
Z Rheumatol. 2023 Dec 29. doi: 10.1007/s00393-023-01454-y. Online ahead of print.ABSTRACTJuvenile dermatomyositis (JDM) is by far the most frequent inflammatory myopathy in childhood and adolescence. It is clinically characterized by inflammatory changes of the skin and muscles but as a multisystemic disease can also affect the skeletal system, the gastrointestinal tract, lungs and heart. Intrinsic (multigenetic risk) and extrinsic factors (triggers) are involved in the pathogenesis resulting in endothelial damage, involvement of fascies, activation of the interferon system and autoimmune reactions including formation of m...
Source: Zeitschrift fur Rheumatologie - December 29, 2023 Category: Rheumatology Authors: Ingrid Georgi Mathias Georgi Johannes-Peter Haas Source Type: research

Protocols in pediatric rheumatology (PROKIND): treat-to-target in polyarticular juvenile idiopathic arthritis
CONCLUSION: Using a treat-to-target approach a dramatic improvement in disease activity, functional capacity and quality of life in polyarticular JIA could be achieved. Even after 12 months an inactive disease was achieved in the majority of cases.PMID:38157052 | DOI:10.1007/s00393-023-01452-0 (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - December 29, 2023 Category: Rheumatology Authors: G Horneff K Minden D Foell J Klotsche K Tenbrock PROKIND-Arbeitsgruppe Source Type: research

Safety and efficacy of telitacicept in refractory systemic lupus erythematosus patients who failed treatment with belimumab : A  case series
CONCLUSION: This is the first case series in SLE patients who accepted telitacicept treatment after failed treatment with belimumab. Our case series and review of the literature show that telitacicept combined with the original standard treatment may significantly improve disease activity while reducing prednisone use. No major safety issues were seen in this group of patients. Telitacicept may be a promising drug for the treatment of refractory lupus hepatitis.PMID:38157053 | DOI:10.1007/s00393-023-01461-z (Source: Zeitschrift fur Rheumatologie)
Source: Zeitschrift fur Rheumatologie - December 29, 2023 Category: Rheumatology Authors: Qiuyu Fan Huiqin Yang Ya Liu Source Type: research

Advances in the diagnostics and treatment of juvenile dermatomyositis
Z Rheumatol. 2023 Dec 29. doi: 10.1007/s00393-023-01454-y. Online ahead of print.ABSTRACTJuvenile dermatomyositis (JDM) is by far the most frequent inflammatory myopathy in childhood and adolescence. It is clinically characterized by inflammatory changes of the skin and muscles but as a multisystemic disease can also affect the skeletal system, the gastrointestinal tract, lungs and heart. Intrinsic (multigenetic risk) and extrinsic factors (triggers) are involved in the pathogenesis resulting in endothelial damage, involvement of fascies, activation of the interferon system and autoimmune reactions including formation of m...
Source: Zeitschrift fur Rheumatologie - December 29, 2023 Category: Rheumatology Authors: Ingrid Georgi Mathias Georgi Johannes-Peter Haas Source Type: research