Tackling respiratory failure in Guillain Barre’ syndrome: Burdens, management and prognosis

Guillain-Barré syndrome (GBS) is a rapidly progressive, inflammatory polyradiculoneuropathy with a heterogeneous presentation and pathogenesis. Diagnosis is clinical and electrophysiological, with supportive features in cerebrospinal fluid. Respiratory insufficiency is a life-threatening manifestation occurring in 20 to 30% of GBS patients associated with poor functional outcome. Progressive weakness of the respiratory muscles is the leading cause of acute respiratory distress, leading to respiratory failure with hypoxemia and hypercapnia. Bulbar weakness may compromise airway patency and predisposes patients to aspiration pneumonia. Mechanical ventilation (MV) is often necessary in patients with severe GBS. Prediction and early recognition of high respiratory failure risk is important to triage patients to the appropriate unit, that is, general ward or intensive care unit (ICU). MV needs to be applied when patients meet respiratory failure criteria and severe clinical signs, as bulbar weakness, ineffective cough and dysautonomia. Prompt initiation of immunotherapy with intravenous immunoglobulin or plasmapheresis, together with supportive care, are effective in GBS. There are challenging clinical questions related to the use of MV in GBS: when to initiate the ventilatory support, what to use, when and how to wean and when to perform tracheostomy. In this review, the authors summarize the up-to-date knowledge of pathophysiology and general management in the real- world clinical practice of the respiratory failure in GBS, focusing on time of MV, of weaning and of tracheostomy.