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diagnosis hematology syndrome treatment lupus dermatology druginduced hemophagocytic hlh lymphohistiocytosis sle summary activation adrenal adultonset antinxp2 antinxp2dm calcifications coagulation common
Drug Induced Lupus vs SLE Drug Induced Lupus (DIL): • Epidemiology: -10% of all lupus cases, drug-dependent,
to 1:1 F:M • Clinical ... NPSLE rare, Malar rash ... 40, F:M 9:1 • Clinical ... Evolution: Chronic disease ... comparison #table #rheumatology
Adult-Onset Still's Disease Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia + Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
Clinical triad ... arthritis, Skin rash ... MAS, Visceral, DIC ... #diagnosis #management ... #treatment #rheumatology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
40, F:M 9:1 • Clinical ... Manifestations: Malar rash ... of SLE versus DIL ... Evolution: Chronic disease ... #Summary #rheumatology
Plain radiography of the abdomen revealed calcification of both adrenal glands. A homozygous mutation in LIPA
deficiency, or Wolman’s disease ... and gut, which causes ... #clincial #peds ... calcifications #infant ... #NEJM #Wolmans
Disseminated Intravascular Coagulation (DIC) Overview Increased Clotting and Consumption of Clotting Factors Findings: • Bleeding • Recent history
organ damage Clinical ... • Severe Liver Disease ... Treat primary cause ... #treatment #management ... #hematology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
fever syndromes Clinical ... Fever is the main clinical ... Petechial or purpuric rash ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
accumulation of clinical ... Assessment • Clinical ... Fibrinogen level, ↑ sIL ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
Evaluation of suspected incomplete Kawasaki Disease 1. AHA consensus recommendations 2. Infants ≤6 months old on day ≥7
Infants ≤6 months ... if they have no clinical ... Albumin ≤3.0 g/dL ... those >day 10 with clinical ... #Diagnosis #Peds
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
to 8 years Clinical ... 100 to 300 mg/dL ... and M panel Management ... DDX - Other causes ... #diagnosis #management
Drug Induced Lupus (DIL) • Epidemiology: -10% of all lupus cases, drug-dependent, 4:1 to 1:1 F:M
to 1:1 F:M • Clinical ... NPSLE rare, Malar rash ... Discontinuation of causal ... #rheumatology # ... diagnosis #treatment #management
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