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syndrome activation antinxp2dm common cvid dermatomyositis differential hypogammaglobulinemia igg4 immunodeficiency immunology macrophage related sjogrens treatment variable workup
IgG4-Related Disease Clinical history: Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor 4 phenotypes: •
Clinical history ... biliary, renal, lung ... concentration PET-CT ... #diagnosis #management ... #treatment #rheumatology
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
overlaps with RA, SLE ... ab • RF • Antinuclear ... MALT Lymphoma • Lung ... Sjogrens #Syndrome #Rheumatology ... #Diagnosis #Management
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
rash) • Heart, lung ... [SLE], AOSD) • ... Activation #Syndrome #Diagnosis ... #Management #Hematology ... #Rheumatology
Vasculitis - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions • Mononeuritis multiplex • Hematuria, proteinuria,
Diagnosis: • Systemic ... , such as SLE, atherosclerotic ... purpura: Strong sign ... ischemia • Skin: Erythematous ... #Rheumatology
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
years Clinical ... lung disease (GLILD ... Chest CT: • Ground-glass ... hypogammaglobulinemia #immunology #hematology ... #diagnosis #management
Anti-NXP-2 Dermatomyositis What? DM with generalized subcutaneous edema Pts have more myalgias, more severe weakness, and an increased prevalence
papules Clinical ... had more severe disease ... #diagnosis #management ... #rheumatology # ... dermatology
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