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Schnitzler's Syndrome: A Diagnostic Consideration in Evaluating the Constellation of Monoclonal Gammopathy and Chronic Urticaria.

Schnitzler's syndrome is a rare clinical entity characterized by intermittent, non-pruritic urticarial rash, fevers, arthralgias, myalgias and monoclonal gammopathy, most commonly of the immunoglobulin M (IgM) subtype. Schnitzler's syndrome should be considered in the differential diagnosis of fever of unknown origin. We report a case of a 56-year-old healthy Caucasian female, who initially presented to the primary care physician's office with complaints of severe generalized fatigue and myalgias involving thighs and calves. Patient subsequently underwent extensive rheumatologic workup, and wa…

The Expanding Field of Secondary Antibody Deficiency: Causes, Diagnosis, and Management.

Antibody deficiency or hypogammaglobulinemia can have primary or secondary etiologies. Primary antibody deficiency (PAD) is the result of intrinsic genetic defects, whereas secondary antibody deficiency may arise as a consequence of underlying conditions or medication use. On a global level, malnutrition, HIV, and malaria are major causes of secondary immunodeficiency. In this review we consider secondary antibody deficiency, for which common causes include hematological malignancies, such as chronic lymphocytic leukemia or multiple myeloma, and their treatment, protein-losing states, and side

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… Arthritis Rheum. (2008) 58:61–72. 10.1002/art.23178 33. Pena-Rossi C, Nasonov E, Stanislav M, Yakusevich V, Ershova O, Lomareva N, et al. … Arthritis Rheum. (2009) 62:64–74. 10.1002/art.25034 113. Tobinai K, Igarashi T, Itoh K, Kurosawa M, Nagai H, Hiraoka A, et al. …

Системный склероз-ассоциированный ILD: Понимание и ограничения ScleroID

Справочная информация/цель: Участие лёгких в системном склерозе (SSc), как правило, оценивается с использованием тестов легочных функций (PFT), CT с высоким разрешением (HRCT) и составных индексов. Результаты, о которых сообщают пациенты (ПРО), включая СклероИД, позволяют получить представление о качестве жизни, однако их связь с клиническими мерами и роль в общей оценке заболеваний остается неясной. Оценить корреляцию между показателями СклероИД и участием легких и активностью/повреждением заболеваний в группе пациентов SSC-ILD из крупного центра третичной медицинской помощи. Методы: Деятельн

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… Arthritis Rheum. 2002;46:2410–2420. doi: 10.1002/art.10486. 24. Khanna D., Tseng C.H., Furst D.E., Clements P.J., Elashoff R., Roth M., Elashoff D., Tashkin D.P. … Rheum. Dis. 2025. in press . 33. …

Neuropathic-like symptoms and central sensitization related signs and symptoms negatively affect the functional performance of patients with knee osteoarthritis - a cross-sectional study.

OBJECTIVE: This study aimed to compare the functional performance among participants with a neuropathic-like symptoms (NS) and central sensitization related signs and symptoms (CS), and their knee osteoarthritis (OA) counterparts. METHODS: A cross-sectional observational study was conducted with 125 participants with knee OA (94 females, mean age 63.1 ​± ​7.4 years). Participants completed a self-reported questionnaire with personal and clinical features and musculoskeletal pain characteristics, including NS (PainDETECT), CS (Central Sensitization Inventory, CSI), and conditioned pain modulat

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… Rheum. Dis. 2014;73:1323–1330. doi: 10.1136/annrheumdis-2013-204763. 5. Nüesch E., Dieppe P., Reichenbach S., Williams S., Iff S., Jüni P. All cause and disease specific mortality in patients with knee or hip osteoarthritis: population based cohort study. … Arthritis Rheum. 1990;33:1601–1610. doi: 10.1002/art.1780331101. 25. Bittencourt J., Bezerra Mp M., et al. Manuscr Submitt Publ; 2020. Cross-cultural Adaptation of the PainDETECT Questionnaire into Brazilian Portuguese Language. 26. Freynhagen R., Tölle T.R., Gockel U., Baron R. …

Burden, Outcome, and Comorbidities of Extrahepatic Manifestations in Hepatitis B Virus Infections.

Hepatitis B virus (HBV) infections affect approximately 296 million people around the world, and the prevalence of any past or present HBV infection during the years 2015-2018 was as high as 4.3%. Acute HBV infection often presents with nonspecific symptoms and is usually self-limited, but 5% of patients can have persistent infections leading to chronic HBV infection and the risk of turning into chronic HBV infection is significantly higher in babies with vertical transmission (95%). Patients with chronic HBV infection are usually asymptomatic, but 15 to 40% of chronic HBV carriers develop cir

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… Arthritis Rheum. 2004;33:355–374. doi: 10.1016/j.semarthrit.2003.10.001. 10. Han H.-x., Su W., Zhou D.-B., Li J., Cao X.-x. Hepatitis B virus-related cryoglobulinemia: Clinical characteristics, virological features, and treatment. … Arthritis Rheum. 2004;51:482–487. doi: 10.1002/art.20401. 20. Arkachaisri T. Serum sickness and hepatitis B vaccine including review of the literature. J. Med. Assoc. Thail. 2002;85((Suppl. 2)):S607–S612. 21. Gupta R., Fakunle I., Samji V., Hale E.B. …

Systemic amyloidosis: an aggressive evolution in a patient with relapsing polychondritis and monoclonal gammopathy of undetermined significance (MGUS) undergoing peritoneal dialysis.

Herein, we report the case of primary amyloidosis with multi-organ involvement in a female patient in her 50s. The patient had a history of relapsing polychondritis, chronic kidney disease, and monoclonal gammopathy of undetermined significance (MGUS). The clinical manifestations included neuropathic pain, sensorimotor polyneuropathy, intrahepatic cholestatic liver injury, gastrointestinal symptoms, dysautonomia, and myocardial thickening. Initial histologic evaluations of the abdominal fat pad aspirate and bone marrow biopsy were negative for amyloid deposition. However, due to a high index o

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… REFERENCES 1 Real de Asúa D Costa R Galván JM Filigheddu MT Trujillo D Cadiñanos J Systemic AA amyloidosis: epidemiology, diagnosis, and management Clin Epidemiol 2014 6 369 377 10.2147/CLEP.S39981 25378951 PMC4218891 2 Hazenberg BP Amyloidosis: a clinical overview Rheum Dis Clin North Am 2013 39 2 … 144 cases Neth J Med 1986 29 11 376 385 3808140 12 Rubinstein SM Stockerl-Goldstein K How to screen for monoclonal gammopathy in patients with a suspected amyloidosis JACC CardioOncol. 2021 3 4 590 593 10.1016/j.jaccao.2021.07.001 34729532 PMC8543087 13 Papa R Lachmann HJ Secondary, AA, Amyloidosis Rheum …

Emerging biologic frontiers for Sjogren's syndrome: Unveiling novel approaches with emphasis on extra glandular pathology.

Primary Sjögren's Syndrome (pSS) is a complex autoimmune disorder characterized by exocrine gland dysfunction, leading to dry eyes and mouth. Despite growing interest in biologic therapies for pSS, FDA approval has proven challenging due to trial complications. This review addresses the absence of a molecular-target-based approach to biologic therapy development and highlights novel research on drug targets and clinical trials. A literature search identified potential pSS treatment targets and recent advances in molecular understanding. Overlooking extraglandular symptoms like fatigue and depr

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… Rheum. Dis. 80 (3), 339–348. 10.1136/annrheumdis-2020-218599 Bai W., Liu H., Dou L., Yang Y., Leng X., Li M., et al. (2022). Pilot study of baricitinib for active Sjogren’s syndrome. Ann. Rheum. Dis. 81 (7), 1050–1052. 10.1136/annrheumdis-2021-222053 Barone F., Bombardieri M., Manzo A., Blades M. … Rheum. Dis. 83 (3), 360–371. 10.1136/ard-2023-224691 Du W., Han M., Zhu X., Xiao F., Huang E., Che N., et al. (2021). The multiple roles of B cells in the pathogenesis of sjogren’s syndrome. Front. …

CAR T-cell therapy in autoimmune diseases: a promising frontier on the horizon.

Although current treatments for autoimmune diseases can effectively control symptoms, they rarely lead to cures and often require lifelong use, accompanied by considerable adverse effects. This emphasizes the urgent need for more targeted therapies that offer long-term efficacy and curative potential. Chimeric antigen receptor (CAR) T-cell therapy presents a promising option by specifically targeting and eliminating autoreactive B cells, with the potential to reset the patient's immune system and promote long-term immune balance. Originally developed for treating hematologic malignancies, wher

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… Semin Arthritis Rheum. (2014) 43:489–97. doi: 10.1016/j.semarthrit.2013.08.003, PMID: 47. Andreakos E, Taylor PC, Feldmann M. Monoclonal antibodies in immune and inflammatory diseases. Curr Opin Biotechnol. (2002) 13:615–20. doi: 10.1016/S0958-1669(02)00355-5, PMID: 48. …

Extraskeletal benefits and risks of calcium, vitamin D and anti-osteoporosis medications.

UnlabelledDrugs used for the prevention and the treatment of osteoporosis exert various favourable and unfavourable extra-skeletal effects whose importance is increasingly recognized notably for treatment selection.IntroductionThe therapeutic armamentarium for the prevention and the treatment of osteoporosis is increasingly large, and possible extra-skeletal effects of available drugs could influence the choice of a particular compound.MethodsThe present document is the result of a national consensus, based on a systematic and critical review of the literature.ResultsObservational research has

The Calcium Conundrum: Hypercalcemia of Unknown Etiology.

Hypercalcemia is a common clinical finding with a wide variety of clinical presentations and etiologies. The differential is broad and includes hyperparathyroidism, malignancy, medication adverse effects, endocrinopathies, and a variety of granulomatous diseases. We present the case of an 84-year-old woman with severe parathyroid hormone-independent hypercalcemia and elevated 1,25-OH Vitamin D. Non-necrotizing granulomas were identified on bone marrow biopsy. After an exhaustive workup for granulomatous disease, the patient was felt to have sarcoidosis isolated to the bone marrow and started o

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… PMC1303627/ 8764626 PMC1303627 9 Granulomatous lesions in bone marrow: clinicopathologic findings and significance in a study of 48 cases Eur J Intern Med Brackers de Hugo L Ffrench M Broussolle C Sève P 468 473 24 2013 23414770 10.1016/j.ejim.2012.11.008 10 Bone and joint sarcoidosis Semin Arthritis Rheum … 2021 34143362 10.1007/s41030-021-00160-x PMC8589889 15 State-of-the-art treatments for sarcoidosis Methodist Debakey Cardiovasc J Papanikolaou IC Antonakis E Pandi A 94 105 18 2022 10.14797/mdcvj.1068 PMC9237819 35772032 16 Clinical characteristics of patients with bone sarcoidosis Semin Arthritis Rheum …

Antibody and aptamer-based therapies for osteoarthritis: Application of antibodies and promise of aptamers.

Osteoarthritis (OA) is a common degenerative inflammatory joint disease with progressive loss of articular cartilage that undermines patients' quality of life. There are no regulatory-approved, disease-modifying OA medications, despite a great deal of studies done to elucidate OA pathogenesis. Until now, OA pharmacological treatment focused mainly on generalized inhibition of inflammation and pain. Currently, monoclonal antibodies and nucleic-acid aptamers emerge as targeted therapies offering potential alternatives by addressing the complex challenges posed by OA, such as specifically reducin

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… Rheum. Dis. 64 2005 682 687 15374852 10.1136/ard.2004.023564 PMC1755481 2 Oliveria S.A. Felson D.T. Reed J.I. Cirillo P.A. Walker A.M. … Rheum. Dis. 60 2001 1070 1073 11602483 10.1136/ard.60.11.1070 PMC1753403 97 Pufe T. Petersen W. Tillmann B. Mentlein R. …

RING-finger type E3 ubiquitin ligase inhibitors as novel candidates for the treatment of rheumatoid arthritis.

Rheumatoid arthritis (RA) significantly affects quality of life. We recently cloned synoviolin, a RING-type E3 ubiquitin ligase implicated in the endoplasmic reticulum-associated degradation (ERAD) pathway. Synoviolin is highly expressed in rheumatoid synovial cells and may be involved in the pathogenesis of RA. Inhibition of synoviolin activity is a potentially useful therapeutic approach for the treatment of RA. We conducted a high-throughput screen of small molecules to find inhibitors of synoviolin autoubiquitination activity. We identified two classes of small molecules, named LS-101 and …

Overview of Ankle Arthropathy in Hereditary Hemochromatosis.

Hereditary hemochromatosis (HH) is an autosomal recessive bleeding disorder characterized by tissue overload of iron. Clinical systemic manifestations in HH include liver disease, cardiomyopathy, skin pigmentation, diabetes mellitus, erectile dysfunction, hypothyroidism, and arthropathy. Arthropathy with joint pain is frequently reported at diagnosis and mainly involves the metacarpophalangeal and ankle joints, and more rarely, the hip and knee. Symptoms in ankle joints are in most cases non-specific, and they can range from pain and swelling of the ankle to deformities and joint destruction.

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… Arthritis Rheum. 2012;64:1697–1707. doi: 10.1002/art.34453. 28. Heiland G.R., Aigner E., Dallos T., Sahinbegovic E., Krenn V., Thaler C., Weiss G., Distler J.H., Datz C., Schett G., et al. Synovial Immunopathology in Haemochromatosis Arthropathy. Ann. Rheum. … Rheum. Dis. 2011;70:563–570. doi: 10.1136/ard.2010.139105. 45. Dejaco C., Stadlmayr A., Duftner C., Trimmel V., Husic R., Krones E., Zandieh S., Husar-Memmer E., Zollner G., Hermann J., et al. …

The Liver in Systemic Illness

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… J Rheum. 2002;29:1576. 65. Maeshima E, Kobayashi T, Mune M, Yukawa S. Systemic lupus erythematosus with haemophagocytosis and severe liver disorder. Ann Rheum Dis. 2002;61:753. doi: 10.1136/ard.61.8.753. 66. Seaman WE, Ishak KG, Plotz PH. … Ann Rheum Dis. 1975;34:198. doi: 10.1136/ard.34.2.198-d. 71. Cimmino MA, Buffrini L, Barisone G. Alkaline phosphatase activity in the serum of patients with rheumatoid arthritis. Z Rheumatol. 1990;49:143. 72. Cimmino MA, Accardo S. …

Osseous Sarcoidosis Mimicking Metastatic Disease in a Patient With Longstanding Pulmonary Sarcoidosis: A Case Report.

Sarcoidosis is a multisystem granulomatous disease in which osseous involvement is uncommon and most often affects the small bones of the hands and feet, whereas axial skeletal disease remains underrecognized and may mimic metastatic malignancy. A 72-year-old man with longstanding pulmonary sarcoidosis and moderate persistent asthma was incidentally found to have multifocal sacral and iliac bone lesions on lumbar spine magnetic resonance imaging (MRI) obtained for evaluation of back pain and radiculopathy. These lesions were radiologically suspicious for osseous metastases, prompting systemic

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… Semin Arthritis Rheum. 2017;47:143–148. doi: 10.1016/j.semarthrit.2017.02.004. 4. Bone marrow involvement in sarcoidosis: an analysis of 50 bone marrow samples. Yanardağ H, Pamuk GE, Karayel T, Demirci S. https://pubmed.ncbi.nlm.nih.gov/12803116/ Haematologia (Budap) 2002;32:419–425. 5. … Semin Arthritis Rheum. 2014;44:371–379. doi: 10.1016/j.semarthrit.2014.07.003. 10. New treatment strategies for pulmonary sarcoidosis: antimetabolites, biological drugs, and other treatment approaches. Baughman RP, Grutters JC. …

Eosinophilic fasciitis (Shulman's disease): review and comparative evaluation of seven patients.

ObjectivesEosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and express fibrogenic cytokines including the transforming factor of tumor growth a and b and interleukins 1 and 6. The purpose of this study was to summarize seven cases of EF in a central hospital.Material and methodsThis was a retrospective and descriptive study of a population with EF of a central hospital. All patients diagnosed with EF in a ho

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… Ann Rheum Dis. 2013;72:1217–1220. doi: 10.1136/annrheumdis-2012-202657. 21. Haddad H, Sundaram S, Magro C, Gergis U. Eosinophilic fasciitis as a paraneoplastic syndrome, a case report and review of the literature. Hematol Oncol Stem Cell Ther. 2014;7:90–92. doi: 10.1016/j.hemonc.2013.12.003. 22. … Semin Arthritis Rheum. 1988;17:221–231. doi: 10.1016/0049-0172(88)90008-x. 30. Espinoza F, Jorgensen C, Pers Y-M. Efficacy of Tocilizumab in the treatment of Eosinophilic fasciitis: Report of one case. Joint Bone Spine. 2015;82:460–461. doi: 10.1016/j.jbspin.2015.02.008. …

The Impact of Educational Interventions on Osteoporosis Knowledge among Korean Osteoporosis Patients.

BACKGROUND: The purpose of this study was to determine the impact of an educational intervention that includes information sharing about absolute fracture risk on the knowledge of osteoporosis and modifiable risk factors among Korean patients with osteoporosis. METHODS: Adults aged >50 years who visited the outpatient clinic for osteoporosis were recruited. Subjects with trauma-related fractures or pathological fractures were excluded. The anthropometric data and clinical risk factors for fracture were collected at baseline. The participants completed the survey questionnaire that measured th

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… Osteoporosis prevention project: a model multidisciplinary educational intervention J Am Osteopath Assoc 2002 102 327 335 12090650 4 Laslett LL Lynch J Sullivan TR Osteoporosis education improves osteoporosis knowledge and dietary calcium: comparison of a 4 week and a one-session education course Int J Rheum … demands for self-management in older adults Geriatr Nurs 2004 25 313 317 15486554 10.1016/j.gerinurse.2004.08.003 13 Kasper MJ Peterson MG Allegrante JP The need for comprehensive educational osteoporosis prevention programs for young women: results from a second osteoporosis prevention survey Arthritis Rheum …

Dynamic thiol and disulphide homoeostasis in fibromyalgia.

BACKGROUND AND PURPOSE: Contrast therapy consists of alternating thermotherapy and cryotherapy repeatedly to assist in the management of acute, subacute, and chronic musculoskeletal conditions. This has been utilized for several decades with good to excellent subjective and objective results reported for patients with swelling (acute to chronic), pain, and loss of motion. Typically, the intervention is performed by either the use of a hot and cold whirlpool or by applying hot and cold packs which can be very time consuming and labor intensive. The purpose of this study was to determine the eff

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… Arthritis Rheum. 1995;38:19–28. doi: 10.1002/art.1780380104. 3. Wolfe F, Smythe HA, Yunus MB, et al. The American College of Rheumatology 1990 criteria for the classification of fibromyalgia. Arthritis Rheum. 1990;33:160–72. doi: 10.1002/art.1780330203. 4. … Int J Rheum Dis. 2017;20:39–45. doi: 10.1111/1756-185X.12550. 10. Sánchez-Domínguez B, Bullón P, Román-Malo L, et al. Oxidative stress, mitochondrial dysfunction and, inflammation common events in skin of patients with Fibromyalgia. …

Metabolomic Profiling in the Characterization of Degenerative Bone and Joint Diseases.

Osteoarthritis and inflammatory arthropathies are a cause of significant morbidity globally. New research elucidating the metabolic derangements associated with a variety of bone and joint disorders implicates various local and systemic metabolites, which further elucidate the underlying molecular mechanisms associated with these destructive disease processes. In osteoarthritis, atty acid metabolism has been implicated in disease development, both locally and systemically. Several series of rheumatoid arthritis patients have demonstrated overlapping trends related to histidine and glyceric aci…

Practical guidance for engaging patients in health research, treatment guidelines and regulatory processes: results of an expert group meeting organized by the World Health Organization (WHO) and the European Society for Clinical and Economic Aspects of Osteoporosis, Osteoarthritis and Musculoskeletal Diseases (ESCEO).

There is increasing emphasis on patient-centred research to support the development, approval and reimbursement of health interventions that best meet patients' needs. However, there is currently little guidance on how meaningful patient engagement may be achieved. An expert working group, representing a wide range of stakeholders and disciplines, was convened by the European Society for Clinical and Economic Aspects of Osteoporosis, Osteoarthritis and Musculoskeletal Diseases (ESCEO) and the World Health Organization (WHO). Through a structured, collaborative process the group generated pract

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… Ann Rheum Dis. 2011;70:722–726. doi: 10.1136/ard.2010.135129. 42. Williamson PR, Altman DG, Bagley H, et al. The comet handbook: version 1.0. Trials. 2017;18:280. doi: 10.1186/s13063-017-1978-4. 43. … Ann Rheum Dis. 2017;76:673–680. doi: 10.1136/annrheumdis-2016-210242. 48. Gladman DD, Mease PJ, Strand V, et al. Consensus on a core set of domains for psoriatic arthritis. J Rheumatol. 2007;34:1167–1170. 49. Tillett W, Eder L, Goel N, et al. …