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Objective To report 5 cases of amyloid arthropathy with joint flexion contracture as the first manifestation,and perform a literature review to improve the understanding of the disease.Methods Retrospective analysis of the clinical manifestations,laboratory and imaging results clinical data of patients with immunoglobulin light chain(AL)amyloidosis related amyloid arthropathy with joints flexion contracture as the first manifestation at the Department of Rheumatology and Immunology,the First Medical Center of People's Liberation Army General Hospital from January 2012 to February 2022.Meanwhile searching the database(CNKI,Wanfang Data,PubMed),excluding myeloma-associated amyloid arthropathy,and analyzing the literature results comprehensively.Results There were a total of 5 patients with amyloid arthropathy with joint flexion contracture as the initial manifestation in this center,including 4 males and 1 female.By February 2022(searching CNKI,Wanfang Data and PubMed),two eligible patients were selected,all males.A total of 7 patients were included with male-to-female ratio of 6:1.The median age was 30-year-old(range from 19 to 73).Spine and limb joints were involved,especially finger joints(7 cases,100%).Soft tissue was involved in 5 patients(71.4%),carpal tunnel syndrome in 4 patients(57.1%),giant tongue in 3 patients(42.9%),shoulder pad sign in 2 patients(28.6%),muscle swelling in 2 patients(28.6%).Five patients were misdiagnosed as rheumatoid arthritis(RA)and 1 patient was misdiagnosed as spondyloarthritis(SpA).The median time of delayed diagnosis was 24 months(2-204 months).The laboratory results showed that 3 patients(42.9%)were positive for immunoelectrophoresis,all of which were λ light chain.Only 1 patient was positive for HLA-B27,while the other patients were negative for autoantibodies and rheumatoid factors.Only 1 patient had elevated C reactive protein(CRP)and erythrocyte sedimentation rate(ESR).Ultrasound examination of the lesion site of 5 patients(71.4%)showed hypoechoic area around the joint,thickening of ligaments or periarticular tenosynovitis.Five patients(71.4%)underwent X-ray examination,of which 4 patients had no bone destruction,1 patient had soft tissue swelling with bone erosion and subchondral cyst.MRI was performed in 5 patients(71.4%),in which low signal on T1 weighted image,medium signal on T2 weighted image and edema signal on peripheral tendon sheath were seen in 1 patient.One patient(14.3%)underwent emission computed tomography(ECT)without abnormal uptake.Conclusion Joint flexion contracture may be the first sign of amyloid arthropathy.Vigilance for amyloid arthropathy is warranted when there is associated soft tissue involvement,negative autoantibodies,and imaging showing no joint destruction but surrounding soft tissue abnormalities.
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It is not clear whether Ca2+-induced Ca2+ release from the sarcoplasmic reticulum (SR) is involved in the regulation of atrial natriuretic peptide (ANP) release. Previously, we have shown that nifedipine increased ANP release, indicating that Ca2+ entry via voltage-gated L-type Ca2+ channel activation decreases ANP release. The purpose of the present study was two-fold: to define the role of SR Ca2+ release in the regulation of ANP release and whether Ca2+ entry via L-type Ca2+ channel is prerequisite for the SR-related effect on ANP release. Experiments were performed in perfused beating rabbit atria. Ryanodine, an inhibitor of SR Ca2+ release, increased atrial myocytic ANP release (8.69+/-3.05, 19.55+/-1.09, 27.31+/-3.51, and 18.91+/-4.76% for 1, 2, 3, and 6microM ryanodine, respectively; all P< 1) with concomitant decrease in atrial stroke volume and pulse pressure in a dose-dependent manner. In the presence of thapsigargin, an inhibitor of SR Ca2+ pump, ryanodine-induced increase in ANP release was not observed. Thapsigargin attenuated ryanodine-induced decrease in atrial dynamic changes. Blockade of L-type Ca2+ channel with nifedipine abolished ryanodine-induced increase in ANP release (0.69+/-5.58% vs. 27.31+/-3.51%; P< 0.001). In the presence of thapsigargin and ryanodine, nifedipine increased ANP release and decreased atrial dynamics. These data suggest that Ca2+-induced Ca2+ release from the SR is inversely involved in the regulation of atrial myocytic ANP release.