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diagnosis management erythematosus hemophagocytic hlh lymphohistiocytosis renal sle syndrome systemic treatment crisis differential eularacr gammopathy hemeonc hepatology hepatorenal hrs mas
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Macrophage Activation ... SJIA], systemic lupus ... Histopathologic criteria ... Diagnosis #Management #Hematology ... #Rheumatology
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus Clinical Domains: • Constitutional domain: Fever • Cutaneous domain: Non-scarring
EULAR/ACR Classification ... Criteria for Systemic ... pericarditis • Hematologic ... low C4, Low C3 and ... Erythematosus #diagnosis #rheumatology
Management of Lupus Nephritis Lupus nephritis (LN) in 10 to 40% of SLE Hallmark = proteinuria 20.5 g/g
Nephritis Lupus ... severity (ISN/RPS classification ... by 12 months Classification ... treatment #management #rheumatology ... #nephrology
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Skin Disease Classification ... Non-autoimmune rheumatologic ... vein thrombosis Hematologic ... Chronic B-cell activation ... Erythematosus #Diagnosis #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
→Activation of CD8 ... IFN-y) → Excessive activation ... of macrophages ... Infections+++: Virus ... treatment #summary #rheumatology
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
rapidly progressing GN ... such as systemic lupus ... LFT • ESR • ANA ... differential #diagnosis #rheumatology ... #classification
Scleroderma Renal Crisis (SRC) Clinical Presentation of Scleroderma Renal Crisis: 1) Acute kidney injury 2) Abrupt onset of hypertension 3)
endothelial cell activation ... confirm diagnosis and ... . pauci-immune GN ... Renal #Crisis #rheumatology ... #nephrology #diagnosis
Hemophagocytic Lymphohistiocytosis (HLH) High mortality without prompt recognition and management. HLH is a critical diagnostic consideration in
termed MAS-HLH: Macrophage ... Activation Syndrome ... Diagnostic Criteria ... of 8 HLH-2004 criteria ... Diagnosis #Management #Hematology
Monoclonal Gammopathy of Renal Significance (MGRS) MGRS • MGRS refers to kidney disease caused by nephrotoxic monoclonal immunoglobulins
does not meet criteria ... guide subtype classification ... Complement pathway activation ... immunotactoid GN ... cryoglobulinemic GN
Hepatorenal Syndrome (HRS) Definition: • Functional, potentially reversible kidney impairment. • Occurs in advanced liver disease: cirrhosis, severe alcoholic
sympathetic + ADH activation ... Diagnosis (Clinical Criteria ... Classification: ... - Salt and ... #Nephrology #Diagnosis
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