Organ Manifestation and Systematic Organ Screening at the Onset of Inflammatory Rheumatic Diseases

Abstract

Background: Inflammatory rheumatic diseases (IRD) are often associated with the involvement of various organs. However, data regarding organ manifestation and organ spread are rare. To close this knowledge gap, this cross-sectional study was initiated to evaluate the extent of solid organ manifestations in newly diagnosed IRD patients, and to present a structured systematic organ screening algorithm. Materials and Methods: The study included 84 patients (63 women, 21 men) with newly diagnosed IRD. None of the patients received any rheumatic therapy. All patients underwent a standardised organ screening programme encompassing a basic screening (including lungs, heart, kidneys, and gastrointestinal tract) and an additional systematic screening (nose and throat, central and peripheral nervous system) on the basis of clinical, laboratory, and immunological findings. Results: Represented were patients with connective tissue diseases (CTD) (72.6%), small-vessel vasculitis (16.7%), and myositis (10.7%). In total, 39 participants (46.5%) had one or more organ manifestation(s) (one organ, 29.7%; two organs, 10.7%; ≥three organs, 6.0%). The most frequently involved organs were the lungs (34.5%), heart (11.9%), and kidneys (8.3%). Lastly, a diagnostic algorithm for organ manifestation was applied. Conclusion: One-half of the patients presented with a solid organ involvement at initial diagnosis of IRD. Thus, in contrast to what has been described in the literature, organ manifestations were already present in a high proportion of patients at the time of diagnosis of IRD rather than after several years of disease. Therefore, in IRD patients, systematic organ screening is essential for treatment decisions.

Diagnosing lung involvement in inflammatory rheumatic diseases—Where do we currently stand?

“…Many IRD present with complex clinical pictures, involving other tissues: In total, 53% of IRD-patients show an organ involvement at initial diagnosis (7) with a predominant affection of the lungs, followed by the heart and kidneys (8)(9)(10)(11)(12)(13). Patients with CTD, myositis/dermatomyositis, and vasculitis are particularly susceptible for solid organ manifestations.…”

“…Pulmonary manifestations present special diagnostic and therapeutic challenges and are associated with a significant morbidity and mortality in IRD-patients. The most common clinical pattern of lung illness encountered in IRD is interstitial lung disease (ILD) ( 8 , 9 , 11 ), ranging between 12.5 and 30.8% at the onset of CTD, 66.7–83.3% with vasculitis and 16.7–100.0% with myositis ( 7 ). In addition, lung diseases are also found in association with rheumatoid arthritis (RA) with a life-time risk of developing ILD of 7.7% ( 14 , 15 ).…”

Clinical Images: Severe interstitial lung disease in Sjögren disease — What happens in the lungs? Inflammation or fibrosis?

“…Moreover, the change of diagnosis was associated with a modification of the therapy in 80% of the patients with CTD‐ILD ( 4 ). Even pulmonary asymptomatic patients with CTD can show ILD, making structured screening essential ( 5 , 6 ). That is why all patients with CTD should receive pulmonary function testing including the quantification of diffusing capacity for carbon monoxide (DLCO) at initial diagnosis.…”

“…That is why all patients with CTD should receive pulmonary function testing including the quantification of diffusing capacity for carbon monoxide (DLCO) at initial diagnosis. In case of reduced DLCO (<80%) or other risk factors, HRCT should be performed to verify CTD‐ILD ( 5 , 6 ). Immunologic bronchoalveolar lavage or cryobiopsy may be considered useful to verify differential diagnosis of CTD‐ILD ( 6 ).…”

“…In case of reduced DLCO (<80%) or other risk factors, HRCT should be performed to verify CTD‐ILD ( 5 , 6 ). Immunologic bronchoalveolar lavage or cryobiopsy may be considered useful to verify differential diagnosis of CTD‐ILD ( 6 ).…”