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diagnosis management differential disease pulmonary syndrome antinxp2 antinxp2dm arteritis autoimmune capillary clarksons classification common comparison cvid dermatology dermatomyositis druginduced gca
IgG4-Related Disease Clinical history: Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor 4 phenotypes: •
IgG4-Related Disease ... head and neck, pancreas ... biliary, renal, lung ... concentration PET-CT ... #rheumatology
Bronchiectasis - Permanent dilation of an airway WHAT CAN YOU SEE ON CT? 1) Lack of tapering of
Bronchiectasis - ... CAN YOU SEE ON CT ... involving both lungs ... Obstruction 6) Rheumatologic ... /Autoimmune diseae
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
• Chronic lung ... Bronchiectasis ... Chest CT: • Ground-glass ... DDX - Other causes ... hypogammaglobulinemia #immunology #hematology
Bronchiectasis - Summary What? • Bronchiectasis is derived from the Greek words bronckos meaning airway and ectasis meaning
Connective tissue diseases ... in the adjacent lung ... of the affected lung ... high-resolution chest CT ... mycobacteria Treatment
Nontuberculous Mycobacteria (NTM) Infections Mycobacterial species other than those belonging to the Mycobacterium tuberculosis complex and
status NTM Cause ... infections Lungs ... Centrilobular nodules - Bronchiectasis ... Hematologic malignancy ... Imaging, high res CT
Drug Induced Lupus vs SLE Drug Induced Lupus (DIL): • Epidemiology: -10% of all lupus cases, drug-dependent,
- ANA > 95% (IgG ... Discontinuation of causal ... life-threatening • Treatment ... Evolution: Chronic disease ... comparison #table #rheumatology
Clarkson’s Disease - Capillary leak syndrome Epidemiology: Roughly 150 published cases, Median age 50 years, No sex
Clarkson’s Disease ... 150 published cases ... Monoclonal gammopathy (IgG ... spacing of IVIg cures ... #rheumatology
Giant cell arteritis (GCA) Giant cell arteritis (GCA) definition: Most common systemic inflammatory vasculitis in older adults
in literature (NEJM ... (Cornelia NEJM ... of the steroid treatment ... Treatment of GCA ... them, but urgent rheumatology
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
Hypocomplementemia • Can cause ... Nephrogenic DI Hematologic ... MALT Lymphoma • Lung ... Adenocarcinoma Treatment ... Sjogrens #Syndrome #Rheumatology
Hodgkin's Lymphoma Overview Hodgkin's lymphoma (HL) is an uncommon hematological malignancy arising from mature B cells. It
Mediastinal mass) • CT ... number of sites of disease ... physical exam, CT ... Hodgkin Lymphoma Treatment ... classification #hematology
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