14 results
Pathophysiology of Hypocomplementemic Urticarial Vasculitis

C1q/anti-C1q antibiody ->
Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Complex Formation - Complement ... TerrierBen #Hypocomplementemic
Causes of Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
C5-C9 Deficiency - Pathogenesis and Clinical Findings
Note:
 • Risk of invasive meningococcal disease is 1000-10000x higher
C5-C9 Deficiency ... higher in C5-C9 deficiency ... is rare Normal complement ... response: The complement ... tissue -> Fatigue, fever
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... Coronary arteries - Fever ... Vasculitis: • Fevers ... against MPO • Complement
Causes of Extravascular and Intravascular Hemolysis - Differential Diagnosis Algorithm
Extravascular - Spleen and RES-mediated hemolysis
 -
Differential Diagnosis Algorithm ... Defects • G6PD deficiency ... • PK deficiency ... perfringens - Complement-Mediated ... Differential #Diagnosis #Algorithm
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
Vasculitis: • Fevers ... and branches • Fever ... Coronary arteries • Fever ... Elevated AST ALT • Hypocomplementemia ... Vasculitis) • Hypocomplementemic
Hemolytic Anemia - Differential Diagnosis Algorithm
 • Sickle cells - Consider sickle cell disease (diverse genotypes):
Differential Diagnosis Algorithm ... ' nucleotidase deficiency ... , Hexokinase deficiency ... bartonella (oroya fever ... Differential #Diagnosis #Algorithm
Complement Deficiency Testing Algorithm
 • Normal CH50 and AH50 AND High suspicion for complement deficiency
 •
Complement Deficiency ... Testing Algorithm ... suspicion for complement ... deficiency • Low ... #Algorithm #hematology
Hemolytic Anemia - Differential Diagnosis Algorithm
Hereditary:
 • Peripheral blood smear + DAT + further History
 •
Differential Diagnosis Algorithm ... DAT (-) → G6PD Deficiency ... Recent travel/Fevers ... Differential #Diagnosis #Algorithm
Complement 1 esterase inhibitor (C1-INH) Deficiency Testing Algorithm
INDICATIONS FOR TESTING:
 • Recurrent angioedema (without urticaria)
 •
Complement 1 esterase ... inhibitor (C1-INH) Deficiency ... Testing Algorithm ... ARUPLabs #C1INH #Complement ... #Testing #Algorithm