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diagnosis rheumatology activation adult antinxp2 antinxp2dm dermatology dermatomyositis differential erythematosus macrophage mas monoclonal onset paraproteinemias sle syndrome systemic
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
• Adult-onset Still ... lupus erythematosus ... erythematosus [SLE], AOSD ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
3rd Decade Skin ... arthritis, adult-onset Still ... Malignancy (e.g. hematologic ... Seizures • Strokes Skin ... La, anti-Jo-1, SCL
Adult Onset Still's Disease Systemic inflammation with urticarial rash. Rash: • Nonpruritic, evanescent (correlates with time of
Adult Onset Still's ... maculopapular skin ... Ferritin >3000 ng/mL ... #disease #AOSD ... #management
Paraproteinemias Entities That Can Feature A Monoclonal Protein/M Component: • MM • WM • MGUS • MGCS • MGRS • Splenic Marginal Zone
• Heavy Chain disease ... Alpha & Mu) Skin ... to cold IgG • Lupus ... Paraproteinemias #Hematology ... Differential #Diagnosis #Oncology
Anti-NXP-2 Dermatomyositis What? DM with generalized subcutaneous edema Pts have more myalgias, more severe weakness, and an increased prevalence
have typical DM skin ... But can still have ... dorsal part of the MCP ... had more severe disease ... Dermatomyositis #diagnosis #management
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