CHICAGO—In systemic lupus erythematosus (SLE), most types of severe pulmonary manifestations are relatively rare, but it’s crucial they be caught because of the potentially dire consequences, said Richard Silver, MD, professor in the Rheumatology Division at the Medical University of South Carolina, Charleston, at the 2018 ACR/ARHP Annual Meeting.
Parenchymal disease—seen in the form of acute lupus pneumonitis, acute respiratory distress syndrome, diffuse alveolar hemorrhage and chronic interstitial pneumonitis—appears in less than 5% of lupus patients. Its mortality rate can run as high as 50%.
“When you see it, you’ve got to recognize it. You’ve got to know you’ve seen it because it can be catastrophic,” Dr. Silver said.
Acute lupus pneumonitis (ALP) usually presents with abrupt fever onset, cough, pleurisy and dyspnea, and is marked by evidence of lupus activity, such as double-stranded DNA antibodies, low C3 and C4, pleural effusions and alveolar infiltrates. The initial concern should be excluding infection, with immunosuppression treatment once infection is ruled out.