Rheumatoid lung disease is a set of changes in the respiratory system that occur in patients with rheumatoid arthritis. It is manifested by chest pain, shortness of breath, cough, fever. There are also signs of the underlying disease: arthralgia, joint stiffness, rheumatoid nodules. Pulmonary changes are detected during lung MSCT, FER, SPECT, biopsy. Additionally, EchoCG and laboratory tests are performed. Drug therapy involves the appointment of corticosteroids, immunosuppressants, mucolytics, oxygen support. Radical treatment includes lung transplantation.
M05.1 Rheumatoid lung disease
Rheumatoid lung disease is one of the most common extra-articular syndromes in rheumatoid arthritis (RA). It occurs in the form of interstitial lung disease, pleurisy, obliterating bronchiolitis, vasculitis, pulmonary hypertension. Rheumatoid lung develops in half of RA patients. Bronchopulmonary lesions cause 10-20% of deaths in this category of patients, which determines the importance of timely detection of rheumatoid-associated diseases in modern pulmonology and rheumatology.
Causes of rheumatoid lung disease
Lung damage in RA is determined both by the autoimmune nature of the disease itself and by concomitant (behavioral, iatrogenic) factors. The causes of the development of rheumatoid arthritis remain largely unclear to date. Among the main theories are the following:
- Hereditary. A significant number of RA patients are found to carry certain alleles of the main histocompatibility complex. It is believed that interstitial lung damage is more associated with HLA-DR2, and bronchial damage is more associated with HLA-DQB1.
- Infectious. It is assumed that autoimmune reactions can trigger various viruses that enter the body and change the genome of the host cell. Among the most likely triggers are herpes viruses, paramyxoviruses, retroviruses, hepatitis B virus.
- Stressful. The immediate triggers triggering the manifestation of RA are various stressors: excessive insolation, hypothermia, toxic effects, endocrine diseases, severe mental shocks and experiences.
Rheumatoid lung is formed in the outcome of the course of rheumatoid arthritis. Additional factors determining the rate and severity of respiratory tract damage are:
- age over 45 years;
- duration of rheumatoid process (more than 5 years);
- RA activity (positivity in the Russian Federation and CCP);
- tobacco smoking (experience >10 years);
- the use of pneumotoxic drugs for the treatment of RA: methotrexate, gold salts, D-penicillamine, TNF inhibitors.
The course of RA is characterized by increased production of antibodies to cyclic citrulline peptide (CCP). The immune complexes formed in this case settle in the target tissues and act as the main damaging factors of connective tissue. First of all, immunocomplex inflammation affects the synovial membrane of the joints, which leads to the development of tendosynovitis, bursitis, and the formation of rheumatoid nodules in periarticular tissues.
It is known that various triggers (cigarette smoke, toxic substances, etc.) cause the activation of the enzyme peptidylarginine deiminase, which triggers the local citrullation of proteins (transformation of arginine into citrulline) with the formation of citrullinated fibrinogen and collagen.
The appearance of citrulline-containing antigens in the respiratory tract initiates the active production of autoantibodies and inflammatory cytokines, which leads to the development of an autoimmune reaction in the respiratory organs. Various viral infections of the upper and lower respiratory tract aggravate inflammation and accelerate the formation of rheumatoid lung.
At the same time, interstitial lung changes prevail in CCP-positive patients, and vascular pathologies prevail in CCP‒negative patients. Bronchoobstructive disorders are closely associated with a decrease in the level of Clara ‒ CC16 cell protein. Rheumatoid nodules (granulomas) in the lungs are more often found against the background of high RF levels in the blood. They have sizes from several 0.5 mm to 5 cm, subpleural location. There is a necrosis zone in the center of the nodules, and decay cavities can subsequently form in their place.
In rheumatoid arthritis, there is a lesion of different parts of the respiratory system. The term “rheumatoid lung” in a broad sense unites the following groups of nosologies:
- Parenchymal: interstitial pneumonia (common, nonspecific, organizing), eosinophilic pneumonia, alveolitis, amyloidosis.
- Bronchial: bronchiolitis (obliterating, follicular, diffuse), bronchiectasis.
- Pleural: dry and effusive pleurisy, pleural fibrosis.
- Vascular: vasculitis, capillaritis, pulmonary hypertension.
Symptoms of rheumatoid lung disease
In patients with RA, joint syndrome comes to the fore: arthralgia, stiffness, joint deformities. Subcutaneous rheumatic nodules are detected in the area of the affected joints. Worries about general weakness, weight loss, subfebrility.
Complaints from respiratory organs are usually nonspecific, often erased. Different forms of rheumatoid lung have similar manifestations. Most patients report shortness of breath during exercise, chronic cough with mucosal or mucopurulent sputum. Patients with bronchiolitis have periodic difficulty breathing. The involvement of the pleura is characterized by chest pain, which increases with breathing and coughing.
In some cases, rheumatoid lung disease is multifaceted with damage to the parenchyma, interstitial tissue, bronchi, bronchioles, pleura. With a simultaneous combination of RA and silicosis, they talk about Kaplan syndrome.
Rheumatoid lesion contributes to the development of structural restructuring of the bronchopulmonary apparatus – pneumofibrosis, bronchiectasis, remodeling of pulmonary vessels. Suppuration of rheumatic nodules (RU) can lead to hydro- and pneumothorax, pulmonary bleeding. 10-20% of patients with rheumatoid arthritis die from late pulmonary complications: hemosiderosis, pulmonary amyloidosis, right ventricular and respiratory failure.
In RA, there is an increased risk of developing opportunistic infections: respiratory tuberculosis, mycobacteriosis, CMV pneumonia, pneumocystis pneumonia, aspergillosis, nocardiosis. There is also a correlation between RA and more frequent development of lung tumors: cancer, non-Hodgkin’s and Hodgkin’s lymphomas.
Diagnostics of rheumatoid lung disease
Patients with respiratory complaints and a history of RA should be consulted by a rheumatologist to assess the activity of the autoimmune process, as well as a pulmonologist to identify the nature and severity of lung damage. Physical examination of patients with rheumatoid lung usually reveals hard breathing, dry wheezing, crepitation and pleural friction noise. Mandatory research:
- Radiation diagnostics. According to MSCT data, various changes are detected in the lungs: areas of the “cellular lung”, “frosted glass”, centrilobular foci, dilation and deformation of the bronchi, RU, thickening of the visceral pleura, pleural effusion, etc.
- Functional research. The analysis of the function of external respiration is performed on the basis of data from spirometry, bodyplethysmography, diffusion test. According to research data, there is a decrease in VCL, bronchoobstructive disorders. Patients with pulmonary hypertension need echocardiography.
- Bronchoscopy. Bronchial endoscopy detects deformation of the bronchial tree, accumulation of sputum. The study allows for diagnostic bronchoalveolar lavage, a transbronchial biopsy for histological examination.
To assess the activity of RA, blood tests for rheumatoid factor, Anti-CCP, and synovial fluid examination are performed. The condition of the musculoskeletal system is studied according to radiography of the hands and feet, affected large joints, ultrasound of the joints.
Changes in the lungs caused by rheumatoid arthritis must be distinguished from similar lesions caused by other causes:
- autoimmune pathologies: dermatomyositis, SLE, systemic scleroderma;
- idiopathic interstitial pneumonia;
- tuberculosis of the lungs;
- bronchioloalveolar cancer;
- pneumoconiosis , etc .
Treatment of rheumatoid lung disease
The treatment of secondary bronchopulmonary changes is not an easy task, since many drugs used for the basic therapy of RA cause lung damage. With a subclinical course of rheumatoid lung and no signs of progression, special therapy is not indicated. With the progression of the pathological process , it is prescribed:
- Drug therapy. It is based on glucocorticosteroids, mycophenolic acid preparations, mucolytics, inhaled bronchodilators and GCS, monoclonal antibodies. When the pressure in the LA increases, vasodilators (calcium antagonists) are added to therapy. In the development of bacterial pneumonia, aminopenicillins, fluoroquinolones, cephalosporins, carbapenems, antimycotic drugs are used.
- Supportive therapy. It includes oxygen therapy, nebulizer therapy, therapeutic gymnastics. Dosed walking, swimming, and sanatorium treatment are useful. As part of the treatment course, it is advisable to conduct plasmapheresis sessions.
- Lung transplantation. With the progression of RF, the question of a donor lung transplant is raised. Transplantation is usually required in patients with pulmonary fibrosis, obliterating bronchiolitis, and pulmonary hypertension.
Prognosis and prevention
Rheumatoid lung disease leads to a decrease in working capacity and quality of life, an increase in the number of hospitalizations. The life expectancy of patients is on average 5-10 years shorter than in the population. More often, patients die from cardiovascular complications, intercurrent infections.
Regular medical examination, comprehensive treatment and pulmonary rehabilitation can slow down the progression of pathological changes in the lungs. It is recommended to give up smoking, vaccination, avoiding the effects of aggressive toxic and physical factors on the body.