July 26, 2019

The Complement Link:  An Intimate Pathophysiological Association Between Rheumatic Disease and ADAMTS-13 Sufficient Thrombotic Microangiopathy

Presenter:  Robert B. Kaplan

Learning Objectives

     At the completion of the program, participants should:

1. Have acquired a general familiarity with the physiology of the complement system and appreciate its role in several complex disorders, including connective tissue disease and thrombotic microangiopathy (TMA)

2. Be able to a) recognize the major clinical features of thrombotic microangiopathy and b) distinguish amongst the unique underlying pathophysiologies of the most common forms.

3. Be cognizant of the general classification schemes of thrombotic microangiopathy and the associated implications for management.

4. Be acquainted with the available evidence that suggests the pathophysiology of select rheumatic diseases and ADAMTS-13 sufficient thrombotic microangiopathy share complement-dependent aspects linking the two disorders, with special attention to the role of complement factor H (CFH) and factor H autoantibodies.

5. Appreciate a) the potential for SLE and scleroderma to trigger exacerbations of TMA in susceptible individuals and b) the propensity for the two diseases to co-exist in the same patient.

6. Have acquired an understanding of the differences in a) patient clinical characteristics b) disease behavior c) response to specific therapies and d) prognosis in patients with lupus nephritis coinciding with thrombotic microangiopathy contrasted with patients with uncomplicated lupus nephritis.

7. Have acquired increased confidence in being able to a) secure an accurate diagnosis early in the disease course and 2) to then select the optimal therapeutic strategy when encountering patients with secondary complement-dysregulated thrombotic microangiopathy (CD-TMA).