Clinical diagnosis
Case 124
【Progress】Because of the long illness term of rheumatoid arthritis and interstitial pneumonia, her activity of daily living was quite low. She was scheduled to take physical training for enabling to stand and walk to toilet by herself.
【Discussion】
In the clinical reality, pulmonary infections and bronchiectasis with rheumatoid arthritis (RA) include nontuberculous mycobacteriosis, bacterial pneumonia, pulmonary tuberculosis, pulmonary aspergillosis, pulmonary cryptococcosis and pneumocystis pneumonia. Steroid and immunosuppressive drugs for RA are related to pulmonary infection; steroid therapy induces pulmonary tuberculosis, bacterial pneumonia; pulmonary aspergillosis; methotrexate therapy induces pneumocystis pneumonia and pulmonary cryptococcosis: TNF alpha inhibitor therapy induces nontuberculous mycobacteriosis and bacterial pneumonia (1, 2).
Meanwhile, interstitial lung disease (ILD) occurs in approximately 30% of the patients with RA (3-6). Of these, 76 % had clinically silent disease (3). ILD is reported to precede arthritis symptoms in 10-20 % (3-6). ILD with RA is not related to drugs but related to production of autoimmune antibodies that include RA factor and anti-cyclic citrullinated peptide (CCP) (2, 3). Higher values of CCP is found in the patients with RA- ILD + RA than in those with RA alone. CCP is considered to induce activation of inflammatory process: tumor necrosis factor (TNF), vascular endothelial growth factor (VEGF), platelet derived growth factor (PDGF) and interleukins (IL), resulting in activation of fibroblasts (3). Smoking is also considered to simulate the same inflammatory process.
As the risk factors of the patients with RA-ILD, smoking, age and male are listed. Although RA occurs in female, RA-ILD typically occurs in male of fifties-sixties decades. Our patient was seventy-year-old female who had never smoked.
Radiological findings on CT in the patients with RA-ILD, include UIP, NSIP and organizing pneumonia. In RA-ILD, UIP pattern occurs in 40-62% and NSIP pattern occurs in 10-32% (3, 7, 8). This is different from other autoimmune diseases such as dermatomyositis, systemic lupus erythematosus, in which NSIP is more predominant than UIP.
The histopathologic characteristic of UIP is subpleural patchy fibrosis and fibroblast foci with abrupt transposition to dense remodeled lung and architectural destruction of the secondary lobule (3-8). The interstitial is infiltrated by lymphocytes, plasma cells, mast cells and eosinophils. Meanwhile, the characteristic of NSIP is interstitial fibrosis or interstitial cellular infiltration of lymphocytes and plasma cells with little or no architectural destruction of secondary lobule (3-8). In our case, chest CT showed honey comb pattern in the basal (lower) lung area, implying UIP.
【Summary】
We present a seventy-year-old female with rheumatoid arthritis related usual interstitial pneumonia. She received home oxygen therapy and steroid + immune-suppressants under diagnosis of rheumatoid lung. It is borne in mind that TNF alpha inhibitor induces nontuberculous mycobacteriosis and bacterial pneumonia, methotrexate induces pneumocystis pneumonia and pulmonary cryptococcosis, and steroid therapy induces pulmonary tuberculosis, bacterial pneumonia; pulmonary aspergillosis. Interstitial lung disease (ILD) occurs in approximately 30% of the patients with RA, that is related to production of autoimmune antibodies that include RA factor and anti-cyclic citrullinated peptide (CCP). CCP activates inflammatory process: tumor necrosis factor (TNF), vascular endothelial growth factor (VEGF), platelet derived growth factor (PDGF) and interleukins (IL), resulting in activation of fibroblasts. The risk factors RA-ILD include smoking, age and male. UIP pattern occurs more than NSIP pattern in RA-ILD, which is different from other autoimmune diseases that NSIP is more predominant than UIP. The histopathologic characteristic of UIP is subpleural patchy fibrosis and fibroblast foci with abrupt transposition to dense remodeled lung and architectural destruction of the secondary lobule. Meanwhile, the characteristic of NSIP is interstitial fibrosis or interstitial cellular infiltration with little or no architectural destruction of secondary lobule.
【References】
1.Takayanagi N et al. Pulmonary infections in patients with rheumatoid arthritis. Nihon Kokyuki Gakkai Zasshi. 2007 Jun;45(6):465-73.
2.Hallowell RW, et al. Interstitial lung disease in patients with rheumatoid arthritis: spontaneous and drug induced. Drugs 2014; 74: 443–450.
3.Shaw M, et al. Rheumatoid arthritis-associated lung disease. European Respiratory Review 2015 24: 1-16; DOI: 10.1183/09059180.00008014
4.O'Dwyer DN, et al. Rheumatoid arthritis (RA) associated interstitial lung disease (ILD). Eur J Intern Med 2013; 24: 597–603.
5.Olson AL, et al. Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med 2011; 183: 372–378.
6.Doyle TJ, et al. A roadmap to promote clinical and translational research in rheumatoid arthritis-associated interstitial lung disease. Chest 2014; 145: 454–463.
7.Gabbay E, et al. Interstitial lung disease in recent onset rheumatoid arthritis. Am J Respir Crit Care Med 1997; 156: 528–535.
8.de Lauretis A, et al. Review series: aspects of interstitial lung disease: connective tissue disease-associated interstitial lung disease: how does it differ from IPF? How should the clinical approach differ? Chron Respir Dis 2011; 8: 53–82.
2018.10.10