This report describes clinical findings of a 28-year old female patient presented with non-healing digit ulcer and Raynaud’s phenomenon. Upon investigation was found to have high eosinophil count alongside hypocomple...This report describes clinical findings of a 28-year old female patient presented with non-healing digit ulcer and Raynaud’s phenomenon. Upon investigation was found to have high eosinophil count alongside hypocomplementemia. This patient was diagnosed with diffuse cutaneous systemic sclerosis and was started on therapy;her other laboratory findings were attributed to a coinciding helminthic infection. This case suggests the possibility of having two different diagnoses presenting at once causing a clinical dilemma.展开更多
The features and treatment of 98 Chinese patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) referred to a single tertiary referring centre were reviewed. Patientsdiagnosed with IgG4-RD according to the c...The features and treatment of 98 Chinese patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) referred to a single tertiary referring centre were reviewed. Patientsdiagnosed with IgG4-RD according to the comprehensive diagnostic criteria (CDC) were includedin the retrospective study from May 2012 to March 2019. We collcted data on clinical, laboratory,imaging, histological features and treatment. Totally, 98 patients with IgG4-RD were enrolled.The common clinical manifestations included abdominal pain, salivary gland swelling andlymphadenopathy. 51% of the patients had multiple organs involvement. Lymph nodes, pancreasand salivary glands were most commonly involved. Four rare sites including ulna, cerebellum,scalp, and mammary gland were found. The serum IgG4 level was increased by 85.7%. The serumIgG4 level was positively correlated with the number of involved organs, IgG and IgG4/IgG. LowC3 and C4 levels were observed in 37.5% and 12.2% patients respectively, and all patients withkidney involvement had hypocomplementemia. A total of 54 patients underwent tissue biopsies,and 55.6%, 31.5% and 11.1% cases were diagnosed as definite, probable and possible IgG4-RD,respectively. Eighty-eight patients received glucocorticoids (GCs) therapy. Five patients underwentradical surgery to remove the lesion. 73% of them presented a complete or partial remission. IgG4-RD is a systemic fibroinflammatory disease with involvement of multiple organs throughout thebody including some rare sites. Most IgG4-RD patients had increased serum IgG4 levels andpatients with kidney involvement showed bypocomplementermia. GCs therapy is effective. Moreresearch is needed to provide a more reliable basis for the diagnosis and treatment of patients.展开更多
文摘This report describes clinical findings of a 28-year old female patient presented with non-healing digit ulcer and Raynaud’s phenomenon. Upon investigation was found to have high eosinophil count alongside hypocomplementemia. This patient was diagnosed with diffuse cutaneous systemic sclerosis and was started on therapy;her other laboratory findings were attributed to a coinciding helminthic infection. This case suggests the possibility of having two different diagnoses presenting at once causing a clinical dilemma.
文摘The features and treatment of 98 Chinese patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) referred to a single tertiary referring centre were reviewed. Patientsdiagnosed with IgG4-RD according to the comprehensive diagnostic criteria (CDC) were includedin the retrospective study from May 2012 to March 2019. We collcted data on clinical, laboratory,imaging, histological features and treatment. Totally, 98 patients with IgG4-RD were enrolled.The common clinical manifestations included abdominal pain, salivary gland swelling andlymphadenopathy. 51% of the patients had multiple organs involvement. Lymph nodes, pancreasand salivary glands were most commonly involved. Four rare sites including ulna, cerebellum,scalp, and mammary gland were found. The serum IgG4 level was increased by 85.7%. The serumIgG4 level was positively correlated with the number of involved organs, IgG and IgG4/IgG. LowC3 and C4 levels were observed in 37.5% and 12.2% patients respectively, and all patients withkidney involvement had hypocomplementemia. A total of 54 patients underwent tissue biopsies,and 55.6%, 31.5% and 11.1% cases were diagnosed as definite, probable and possible IgG4-RD,respectively. Eighty-eight patients received glucocorticoids (GCs) therapy. Five patients underwentradical surgery to remove the lesion. 73% of them presented a complete or partial remission. IgG4-RD is a systemic fibroinflammatory disease with involvement of multiple organs throughout thebody including some rare sites. Most IgG4-RD patients had increased serum IgG4 levels andpatients with kidney involvement showed bypocomplementermia. GCs therapy is effective. Moreresearch is needed to provide a more reliable basis for the diagnosis and treatment of patients.
