Abstract
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.
- © 2014 ERS
