Table 1.
DEFICIENCIES OF COMPLEMENT COMPONENTS |
Increased susceptibility to bacterial infections |
Repeated severe (invasive) bacterial infections |
Meningococcal disease |
Autoimmune disease (SLE and related diseases) |
HAE (C1-INH deficiency) |
AAE (C1-INH deficiency) |
DISEASE ACTIVITY AND DIFFERENTIAL DIAGNOSIS |
SLE |
Antiphospholipid syndrome |
Urticarial vasculitis |
Cryoglobulinemia |
Various (RA, GPA, Henoch Schönlein) |
C3 glomerulopathy and other types of glomerulonephritis |
Thrombotic microangiopathies (aHUS) |
Transplantation (AMR) |
ASSESSMENT OF THERAPEUTIC EFFECTS |
C1-INH |
Eculizumab |
SLE, systemic lupus erythematosus; HAE, hereditary angioedema,; AAE, acquired angioedema; C1-INH, C1 inhibitor; RA, rheumatoid arthritis; GPA, granulomatous polyangiitis; antibody mediated rejection; aHUS, atypical haemolytic uremic syndrome.