CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY: KONSEP TERKINI DALAM PATOFISIOLOGI

Abstrak

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an autoimmune disorder characterized by progressive or relapsing symmetric proximal and distal weakness and sensory deficits lasting over 8 weeks. Its pathophysiology is complex and heterogeneous, involving both cellular immunity (T-cell and macrophage infiltration across the blood-nerve barrier, causing demyelination via cytokines and phagocytosis) and humoral immunity, including complement activation. While macrophage-induced demyelination is a key feature, the specific autoantibody triggers are not fully identified. The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines classify CIDP into typical and variant forms, which exhibit different patterns of demyelination reflecting a variable dominance of cellular versus humoral immune responses. Recent research highlights autoantibodies against nodal/paranodal proteins (e.g., neurofascin-155, contactin-1). Patients with these antibodies often have distinct clinical phenotypes and a partial response to intravenous immunoglobulin (IVIg), leading to their reclassification in the guidelines as separate "autoimmune nodopathies." This refined understanding of CIDP's diverse immunopathogenesis is crucial for developing more specific diagnostic and therapeutic strategies.

Keywords: CIDP, demyelination, immunopathogenesis, EFNS/PNS diagnostic criteria, autoimmune nodopathy