RT Journal Article
SR Electronic
T1 Early introduction of non invasive ventilation (NIV) in patients with systemic lupus erythematosus (SLE) and diaphragmatic weakness
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P451
VO 44
IS Suppl 58
A1 Ziaudeen Ansari
A1 Pooja Sharma
A1 Susil Herath
A1 Mike Lang
A1 Amina Mohamed
A1 Nazim Nathani
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P451.abstract
AB Background : Respiratory failure and domiciliary NIV requirement is not well defined in patient with SLE and respiratory muscle weakness. Patients with SLE can develop ventilatory failure from muscle weakness due to a combination of myopathy and vasculitis induced neuromuscular compromise.Our NIV service caters to a national referral Centre for lupus. We have come across 6 SLE patients within last 4 years who presented with respiratory compromise. The predominant cause of respiratory compromise seemed to be significant and progressive diaphragmatic weakness.Case 1: 31 years female who had diaphragmatic weakness, which was diagnosed at early stage and substantially reversed with early aggressive immunosuppression and currently stable on NIV.Case 2: 41 years female developed respiratory sepsis within one month of NIV introduction and has required longterm tracheostomy and remains ventilated.Case 3: 46 years female succumbed to global and progressive respiratory weakness while on NIV and died.Case 4: 79 years male with neuromuscular weakness died within few days of initiating NIV.Case 5: 46 years female on NIV for last 24 months doing well with stable lung function.Case 6: 67 years male on NIV for 31 months doing well with stable lung function.Conclusion; SLE related diaphragmatic myopathy seems to have a reversible component and needs to be aggressively managed. NIV is effective in managing ventilatory impairment in shrinking lungs. Respiratory and rheumatology pathway needs to be developed with a low threshold in investigating and managing lupus patients with early signs of respiratory impairment.