The patient had no obvious pulmonary symptoms, but a routine chest X-ray helped investigators arrive at the diagnosis.
A case report in which a patient with systemic lupus erythematosus (SLE) was found to have lymphocytic interstitial pneumonia (LIP) despite a lack of obvious symptoms, underscores the potential for severe pulmonary complications in patients with SLE.
In an article in the Polish Archives of Internal Medicine, corresponding author Magdalena Roszkowska, MD, of Poland’s National Institute of Geriatrics, Rheumatology and Rehabilitation, and colleagues reported on the case of a 30-year-old who arrived in a hospital rheumatology department with a host of symptoms and was eventually diagnosed with LIP as a manifestation of SLE.
The investigators noted that the patient was unique because, while more than half of patients with SLE have pulmonary manifestations, those manifestations rarely include LIP. More typical are conditions like pleurisy, pulmonary hypertension and pulmonary embolism, among others.
In this case, the patient reported a fever, weight loss, arthritis, myalgia, nonscarring alopecia, photosensitivity, butterfly rash, and oral ulcers, as well as xerostomia and eye dryness, Roszkowska and colleagues wrote.
“Laboratory tests revealed elevated inflammatory markers (C-reactive protein and erythrocyte sedimentation rate), hypergammaglobulinemia, and presence of rheumatoid factor and antinuclear antibodies at a titer of 1:10 240 with Sjögren's-syndrome-related antigen A, Sjögren's syndrome type B, and ribosomal P antibodies,” the authors reported.
Hepatitis B, hepatitis C, and HIV were all ruled out through diagnostic testing. However, the patient met the criteria to be diagnosed with SLE and Sjögren’s syndrome.
Treating the patient proved challenging. His doctors began with intravenous methylprednisolone followed by a tapered dose of prednisone. They next tried to prescribe disease-modifying antirheumatic drugs (DMARDs), including methotrexate, azathioprine, cyclosporine, and hydroxychloroquine, but all were either ineffective or, in the case of methotrexate, led to adverse effects.
Six months after his first visit to the clinic, the patient was re-admitted with severe erythematosus-infiltrative skin lesions that covered more than 70% of the surface of his skin, and which appeared after 30 minutes of exposure to sunlight. His physicians again turned to glucocorticoids and DMARDs. A combination of high-dose glucocorticoids, intravenous immunoglobulins, and 2 g/d of mycophenolate mofetil appeared to lead to improvement in the patient. However, despite the apparent progress, doctors discovered something unexpected during a routine chest X-ray: emphysematous bulla.
Although an arterial blood gas measurement did not show abnormalities, a battery of other pulmonary tests revealed obstruction and impairment. High-resolution computed tomography revealed multiple cysts and ground-glass opacities consistent with LIP.
The typical first-line therapy for LIP is oral glucocorticoids, and Roszkowska wrote that the patient’s rheumatologists and pulmonologists decided to continue treatment with glucocorticoids and mycophenolate mofetil. The patient was released and tracked in an outpatient setting.
Roszkowska and colleagues noted that the reported 5-year survival rate for patients with LIP is around 50-67%. In about 5% of cases, LIP leads to lymphoma, the authors added.
The investigators said the case is a reminder of the importance of vigilance when it comes to pulmonary manifestations of SLE.
“Our patient did not present any pulmonary symptoms, despite advanced abnormalities on imaging,” they concluded. “For this reason, we suggest that all patients diagnosed with SLE (even asymptomatic) should be screened for pulmonary complications.”
Roszkowska M, Nowakowska-Płaza A, Wroński J, Płaza M, and Zielińska A. Lymphocytic interstitial pneumonia as an extremely rare manifestation of systemic lupus erythematosus. Pol Arch Intern Med. Published online October 11, 2021. doi:10.20452/pamw.16115