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diagnosis differential erythematosus hematology lupus sle symptoms classification management myositis scleroderma sclerosis vasculitis champs common complement criteria cvid eularacr hypocomplementemia
Systemic Sclerosis (Scleroderma) Multi-system autoimmune disease characterized by vasculopathy and progressive fibrosis of skin and internal organs 3
autoimmune disease ... skin thickening Signs ... : scleroderma renal ... Scleroderma #SSc #rheumatology ... #diagnosis #signs
Systemic Sclerosis (Scleroderma) Limited - CREST: • Calcinosis • Raynaud phenomenon • Esophageal dysmotility • Sclerodactyly •
Systemic Sclerosis ... • Scleroderma renal ... @AnnKumfer #Systemic ... Scleroderma #diagnosis #signs ... #symptoms #rheumatology
Pancytopenia - Differential Diagnosis Congenital Diseases: • GATA2 deficiency • Fanconi’s anemia • Wiskott-Aldrich syndrome • Ribosomopathy or telomeropathy Cancer: • Leukemia • Lymphoma Inflammatory
Inflammatory and Autoimmune ... Diseases: • Systemic ... Cytomegalovirus • Epstein-Barr ... Differential #Diagnosis #Hematology
Autoimmune Myositis Necrotizing myopathy is associated with higher CK levels, greater level of muscle weakness, less systemic
Autoimmune Myositis ... weakness, less systemic ... heliotrope rash, Shaw sign ... , holster sign, ... Myositis #diagnosis #rheumatology
Clinical Features of Systemic Lupus (SLE) General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%) Eye: Sjögrens (15%) Skin
Clinical Features of Systemic ... joints Nervous system ... Abdominal pain (20%) Renal ... erythematosus #signs ... symptoms #diagnosis #rheumatology
Causes of Hypocomplementemia - CHAMPS Mnemonic C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis H - Heavy Chain deposition
Atypical HUS, autoimmune ... glomerulonephritis (>90%) S - Systemic ... Erythematosus (total 50, renal ... #differential #hematology
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus Clinical Domains: • Constitutional domain: Fever • Cutaneous domain: Non-scarring
Classification Criteria for Systemic ... Thrombocytopenia, Autoimmune ... hemolysis • Renal ... Criteria #SLE #Systemic ... Erythematosus #diagnosis #rheumatology
Common Variable Immunodeficiency (CVID) What? CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
: Pneumonia • Autoimmune ... thrombocytopenia and autoimmune ... hemolytic anemia) • Systemic ... Chronic infections (Epstein-Barr ... hypogammaglobulinemia #immunology #hematology
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
pulmonary and renal ... central nervous system ... purpura: strong sign ... • Urinalysis (Renal ... differential #diagnosis #rheumatology
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
be any age) Autoimmune ... Central nervous system ... cirrhosis: 3%-20% Renal ... Distal (Type I) renal ... Sjogrens #Syndrome #Rheumatology
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