Pulmonary Complications of Systemic Lupus Erythematosus

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease characterized by the production of pathogenic autoantibodies and immune complexes and is responsible for significant morbidity and mortality through a wide range of clinical manifestations which can affect almost any organ. Pulmonary involvement is prevalent and seen in 50 to 70% of SLE patients and may even be the presenting feature in 4 to 5% of patients. By 10 years postdiagnosis, 12% will have accumulated an element of permanent lung damage. Pulmonary complications are broad and include pleural disease, interstitial lung disease (ILD), vasculitis, pulmonary embolism, pulmonary hypertension, large airway disease, shrinking lung syndrome, and infection. Conditions can range mostly from asymptomatic, for example, in mild cases of pleural effusion or obstructive airway disease, to life-threatening disease, for example, in acute lupus pneumonitis or diffuse alveolar hemorrhage. ILD and pulmonary hypertension are both frequently seen in other autoimmune rheumatic diseases such as systemic sclerosis; however, in SLE, they tend to be milder and have a comparatively favorable prognosis. Although collectively pulmonary involvement in SLE is common, the heterogeneity of SLE and rareness of individual complications make clinical trials difficult and treatment is usually based on case series reports and anecdotal experience with various immunosuppressive agents. Some of these immunosuppressive agents such as azathioprine, methotrexate, and cyclophosphamide have also been linked with drug-induced lung injury.

Association of anti-Ro52 autoantibodies with interstitial lung disease in connective tissue diseases

“…Among CTDs, idiopathic inflammatory myopathy (IIM) was the most often seen underlying disease (85.4%), followed by undifferentiated connective tissue disease (UCTD), systemic sclerosis, rheumatoid arthritis (RA) and primary Sjögren’s syndrome (pSS). As for systemic lupus erythematosus, only 6.5% anti-Ro52 positive patients presented ILD, consistent with its low incidence in this prototypic autoimmune disease 4…”

Systemic Lupus Erythematosus triggered by SARS-CoV-2 Infection: A Systematic Review Based on a Case Report

“…These symptoms are nonspeci c, and therefore do not allow any differentiation between these diseases. Pulmonary manifestations are very common in patients with SLE, with 50% to 70% of patients suffering from some form of pulmonary complications during the disease process [21]. However, acute pulmonary pneumonitis is an uncommon (1-2%) manifestation of SLE (although, in approximately half of the patients with acute lupus pneumonitis, pneumonitis is the rst manifestation of SLE) [21,22].…”

“…Pulmonary manifestations are very common in patients with SLE, with 50% to 70% of patients suffering from some form of pulmonary complications during the disease process [21]. However, acute pulmonary pneumonitis is an uncommon (1-2%) manifestation of SLE (although, in approximately half of the patients with acute lupus pneumonitis, pneumonitis is the rst manifestation of SLE) [21,22]. In one case series, the most common presenting symptoms of lupus pneumonitis included fever, cough, dyspnea, hypoxia, and lung crepitations [23].…”

“…The main respiratory manifestations of COVID-19 are cough (57%), dyspnea (23%) and chest pain (7%) [19]. Both diseases showed a ground glass appearance by CT [21,24]. Due to these similarities, and given the current pandemic state by SARS-CoV-2, respiratory symptoms should be attributed to COVID-19 unless the patient does not respond to empiric treatment for pneumonia and when extrapulmonary features of SLE are noted (e.g., malar rash, oral ulcers, alopecia, polyserositis, abnormal urine sediment, renal insu ciency, cytopenia, thrombophilia, lymphadenopathy, splenomegaly, joint swelling).…”

Neue Klassifikationskriterien des SLE: was helfen sie für die klinische Diagnose des SLE?

“…Andere seltene Manifestationen betreffen fast alle Organsysteme. Es gibt die Lupuspneumonitis und die interstitielle Lungenerkrankung beim SLE [32], die Lupus-Myositis [33], die Lupus-Hepatitis und die Lupus-Pankreatitis [34] und so ungewöhnliche Manifestationen wie interstitielle Zystitis [35] oder Hyperkalzämie [36]. Besonders mehrere solcher seltener Manifestationen würden die Wahrscheinlichkeit eines SLE deutlich erhöhen, auch wenn die Kriterien-Manifestationen fehlen.…”