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differential activation capillary clarksons classification common conditions cvid extrarticular hypogammaglobulinemia immunodeficiency immunology joint leak macrophage mas pain phenotypes polychondritis relapsing
Aortitis - Differential Diagnosis Framework Non-Infectious Aortitis: • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
Aortitis: • Vasculitides ... arteritis, Cogan's Syndrome ... : IgG4-Related Disease ... erythematosus (SLE ... #rheumatology
IgG4-Related Disease Clinical history: Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor 4 phenotypes: •
IgG4-Related Disease ... Clinical history ... biliary, renal, lung ... #diagnosis #management ... #rheumatology
Extra-articular and Other Conditions Associated with Joint Pain Eyes • Iritis or uveitis - Spondyloarthropathies, Sarcoidosis,
• Interstitial lung ... , SLE • Pulmonary ... Vasculitis (e.g. ... #differential #diagnosis ... #rheumatology
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
to 8 years Clinical ... lung disease (GLILD ... Chest CT: • Ground-glass ... hypogammaglobulinemia #immunology #hematology ... #diagnosis #management
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
inflammatory lesions-interstitial ... to exclude are infections ... claudication, aortitis ... , and SLE) • Urinalysis ... #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 ... salivary ducts Clinical ... MALT Lymphoma • Lung ... #Rheumatology # ... Diagnosis #Management
Clarkson’s Disease - Capillary leak syndrome Epidemiology: Roughly 150 published cases, Median age 50 years, No sex
Monoclonal gammopathy (IgG ... filling +++ (risk of pulmonary ... of infections) ... #diagnosis #management ... #rheumatology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
rash) • Heart, lung ... erythematosus [SLE ... ], AOSD) • Infection ... #Diagnosis #Management ... #Hematology #Rheumatology
TAFRO TAFRO syndrome was first described in 2010, standing for: - Thrombocytopenia - Anasarca - Fever - Reticulin fibrosis - Organomegaly TAFRO syndrome
erythematosus (SLE ... ANCA-associated vasculitis ... - IGG4 - Hepatic ... Hypoalbuminemia Treatment ... #management #rheumatology
Relapsing Polychondritis What is it? Recurrent inflammation of the cartilage in the body (Autoimmune disorder) Who? • Most frequently: 40
cases such as: • SLE ... ○ Tuberculosis ... affected tissue Treatment ... Polychondritis #rheumatology ... #diagnosis #management
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