Rheumatoid lung disease

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Rheumatoid lung disease
Other namesRheumatoid lung
Specialty Rheumatology

Rheumatoid lung disease is a disease of the lung associated with RA, rheumatoid arthritis. Rheumatoid lung disease is characterized by pleural effusion, pulmonary fibrosis, lung nodules and pulmonary hypertension. Common symptoms associated with the disease include shortness of breath, cough, chest pain and fever. It is estimated that about one quarter of people with rheumatoid arthritis develop this disease, which are more likely to develop among elderly men with a history of smoking. [1] [2]

Contents

Rheumatoid lung is separate from but often associated with Interstitial lung disease(ILD).

Signs and symptoms

Most common:

People may not present with all these symptoms or non at all.

From most to lest common:

Causes

The exact cause of rheumatoid lung disease is unknown. However, associated factors could be due largely to smoking. Sometimes, the medicines used to treat rheumatoid arthritis, especially methotrexate, may result in lung disease. [4]

Prevention:

Mechanism

RA is a complex and poorly understood disease.

However, the characteristic presence of antibodies to citrullinated proteins (anti-CCP) suggests that there are pathogenic mechanisms unique to RA. Recent studies suggest a link between smoking, HLA-DRBSE, anti-CCP and RA. Smoking has long been linked to RA and worsens articular disease as well as rheumatoid lung disease. Exposure to cigarette smoke leads to the activation of PADI enzymes that deaminate arginine to citrulline, producing autoantigens like citrullinated collagen and fibrinogen. Autoantigenic peptides containing citrulline residues are preferentially bound by HLA-DRB SE proteins and are presented to T cells, which interact with CCP-specific B cells found at sites containing autoantigen and produce inflammatory cytokines and autoantibodies, which accelerate disease. Bacterial or viral infections of the respiratory tract may also exacerbate pulmonary inflammation and rheumatoid lung disease. [5]

Diagnosis

The diagnosis of RA was formerly based on detection of rheumatoid factor (RF). However, RF is also associated with other autoimmune diseases. The detection of anti-CCP is currently considered the most specific marker of RA. The diagnosis of rheumatoid lung disease is based on evaluation of pulmonary function, radiology, serology and lung biopsy. High resolution CT scans are preferred to chest X-rays due to their sensitivity and specificity. [6]

Associated doctors to diagnosis this properly would be a Rheumatologists or Pulmonologist.

Within a physical examination doctors could find possible indications, such as hearing crackles (rales) when listening to the lungs with a stethoscope. Or, there may be decreased breath sounds, wheezing, a rubbing sound, or normal breath sounds. When listening to the heart, there may be abnormal heart sounds. Bronchoscopic, video-assisted, or open lung biopsy allows the histological characterization of pulmonary lesions, which can distinguish rheumatoid lung disease from other interstitial lung diseases. [6]

The following tests may also show signs of rheumatoid lung disease: [7]

Treatment

Many people with this condition have no symptoms. Treatment is aimed at the health problems causing the lung problem and the complications caused by the disorder.[ citation needed ]

Fast-acting drugs for RA include aspirin and corticosteroids, which alleviate pain and reduce inflammation. Slow-acting drugs termed disease modifying antirheumatic drugs (DMARDs), include gold, methotrexate and hydroxychloroquine (Plaquenil), which promote disease remission and prevent progressive joint destruction. In patients with less severe RA, pain relievers, anti-inflammatory drugs and physical rest are sufficient to improve quality of life. In patients with joint deformity, surgery is the only alternative for recovering articular function. [6]

Prognosis is related to the underlying disorder and the type and severity of lung disease. In severe cases, lung transplantation can be considered. This is more common in cases of bronchiolitis obliterans, pulmonary fibrosis, or pulmonary hypertension. Most complications are not fatal, but does reduce life expectancy to an estimated 5 to 10 years. [8]

Epidemiology

The prevalence of RA is around 0.3–1.2% (0.92% of Americans). Women are 2–3 times more susceptible than men. The prevalence of rheumatoid lung disease in patients with RA depends on the method used for diagnosis: chest X rays (5%), high resolution CT scans (10–40%). [6]

A study showed 582 patients with RA and 603 subjects without RA were followed for a mean of 16.4 and 19.3 years, respectively. The lifetime risk of developing ILD was 7.7% for RA patients and 0.9% for subjects without RA. The risk of developing ILD was higher in patients with older age at RA onset, among male patients and for individuals with parameters that indicate more severe RA.

Survival of RA patients diagnosed with ILD was worse compared to RA patients without ILD. ILD contributed approximately 13% to the excess mortality of patients with RA patients when compared to the general population. [9]

History

Rheumatoid Lung was first described in 1948.[ citation needed ]

1948, they published several cases of patients with RA who had severe erosive joint disease who also developed an interstitial lung and suggested there may be an association between the inflammatory joint disease and interstitial lung disease.[ citation needed ]

1953, Anthony Caplan described rheumatoid nodules within the lung parenchyma, associated with pneumoconiosis in coal miners, who were exposed to coal dust. [10]

1954, rheumatoid lung nodules were found in patients with RA who were not exposed to coal dust and without pneumoconiosis.

1955 there was a short case series of about 10 patients with RA whose autopsies showed that the pleural disease was much higher in rheumatoid patients than in the general population, and much higher than what they had previously seen clinically.

1961, Cudkowicz described the first pulmonary function tests and lung biopsies were done in RA patients. [11]

Research

According to a recent study, the main risk factors for RA-ILD are advancing age, male sex, greater RA disease activity, rheumatoid factor (RF) positivity, and elevated titers of anticitrullinated protein antibodies such as anticyclic citrullinated peptide. Cigarette smoking also appears to increase risk of RA-ILD, especially in patients with human leukocyte antigen DRB1.[ citation needed ]

A recently published retrospective study by a team from Beijing Chao-Yang Hospital in Beijing, China, supported three of the risk factors listed for RA-ILD and identified an additional risk factor. In that study of 550 RA patients, logistic regression analysis of data collected on the 237 (43%) with ILD revealed that age, smoking, RF positivity, and elevated lactate dehydrogenase closely correlated with ILD.Recent studies have identified risk factors for disease progression and mortality. A retrospective study of 167 patients with RA-ILD determined that the usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) was a risk factor for progression, as were severe disease upon diagnosis and rate of change in pulmonary function test results in the first 6 months after diagnosis.

A study of 59 RA-ILD patients found no median survival difference between those with the UIP pattern and those without it. But the UIP group had more deaths, hospital admissions, need for supplemental oxygen, and decline in lung function. [12]

Related Research Articles

Pleural cavity Thin fluid-filled space between the two pulmonary pleurae (visceral and parietal) of each lung

The pleural cavity, pleural space, or interpleural space, is the potential space between the pleurae of the pleural sac that surrounds each lung. A small amount of serous pleural fluid is maintained in the pleural cavity to enable lubrication between the membranes, and also to create a pressure gradient.

Pleural effusion Accumulation of excess fluid in the pleural cavity

A pleural effusion is accumulation of excessive fluid in the pleural space, the potential space that surrounds each lung. Under normal conditions, pleural fluid is secreted by the parietal pleural capillaries at a rate of 0.01 millilitre per kilogram weight per hour, and is cleared by lymphatic absorption leaving behind only 5–15 millilitres of fluid, which helps to maintain a functional vacuum between the parietal and visceral pleurae. Excess fluid within the pleural space can impair inspiration by upsetting the functional vacuum and hydrostatically increasing the resistance against lung expansion, resulting in a fully or partially collapsed lung.

Radiology (X-rays) is used in the diagnosis of tuberculosis. Abnormalities on chest radiographs may be suggestive of, but are never diagnostic of TB, but can be used to rule out pulmonary TB.

Interstitial lung disease Group of diseases

Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory diseases affecting the interstitium of the lungs. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process is disrupted, and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months. The average rate of survival for someone with this disease is between three and five years. The term ILD is used to distinguish these diseases from obstructive airways diseases.

Chest radiograph Projection X-ray of the chest

A chest radiograph, called a chest X-ray (CXR), or chest film, is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common film taken in medicine.

Pulmonary fibrosis Human disease

Pulmonary fibrosis is a condition in which the lungs become scarred over time. Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing. Complications may include pulmonary hypertension, respiratory failure, pneumothorax, and lung cancer.

Cryptogenic organizing pneumonia Medical condition

Cryptogenic organizing pneumonia (COP), formerly known as bronchiolitis obliterans organizing pneumonia (BOOP), is an inflammation of the bronchioles (bronchiolitis) and surrounding tissue in the lungs. It is a form of idiopathic interstitial pneumonia.

Respiratory disease Disease of the respiratory system

Respiratory diseases, or lung diseases, are pathological conditions affecting the organs and tissues that make gas exchange difficult in air-breathing animals. They include conditions of the respiratory tract including the trachea, bronchi, bronchioles, alveoli, pleurae, pleural cavity, the nerves and muscles of respiration. Respiratory diseases range from mild and self-limiting, such as the common cold, influenza, and pharyngitis to life-threatening diseases such as bacterial pneumonia, pulmonary embolism, tuberculosis, acute asthma, lung cancer, and severe acute respiratory syndromes, such as COVID-19. Respiratory diseases can be classified in many different ways, including by the organ or tissue involved, by the type and pattern of associated signs and symptoms, or by the cause of the disease.

Bronchiolitis obliterans Medical condition

Bronchiolitis obliterans (BO), also known as obliterative bronchiolitis, constrictive bronchiolitis and popcorn lung, is a disease that results in obstruction of the smallest airways of the lungs (bronchioles) due to inflammation. Symptoms include a dry cough, shortness of breath, wheezing and feeling tired. These symptoms generally get worse over weeks to months. It is not related to cryptogenic organizing pneumonia, previously known as bronchiolitis obliterans organizing pneumonia.

Mixed connective tissue disease commonly abbreviated as MCTD, is an autoimmune disease characterized by the presence of elevated blood levels of a specific autoantibody, now called anti-U1 ribonucleoprotein (RNP) together with a mix of symptoms of systemic lupus erythematosus (SLE), scleroderma, and polymyositis. The idea behind the "mixed" disease is that this specific autoantibody is also present in other autoimmune diseases such as systemic lupus erythematosus, polymyositis, scleroderma, etc. MCTD was characterized as an individual disease in 1972 by Sharp et al., and the term was introduced by Leroy in 1980.

Caplan's syndrome is a combination of rheumatoid arthritis (RA) and pneumoconiosis that manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray.

Idiopathic pulmonary fibrosis Medical condition

Idiopathic pulmonary fibrosis (IPF) is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue. It is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired, and abnormally large and dome shaped finger and toenails. Complications may include pulmonary hypertension, heart failure, pneumonia or pulmonary embolism.

Usual interstitial pneumonia Medical condition

Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. The scarring (fibrosis) involves the pulmonary interstitium. UIP is thus classified as a form of interstitial lung disease.

High-resolution computed tomography Diagnostic imaging test

High-resolution computed tomography (HRCT) is a type of computed tomography (CT) with specific techniques to enhance image resolution. It is used in the diagnosis of various health problems, though most commonly for lung disease, by assessing the lung parenchyma. This article deals with high-resolution computed tomography of the lungs or HRCT Chest.

Rheumatoid nodule Medical condition

A rheumatoid nodule is a lump of tissue, or an area of swelling, that appear on the exterior of the skin usually around the olecranon or the interphalangeal joints, but can appear in other areas. There are four different types of rheumatoid nodules: subcutaneous rheumatoid nodules, cardiac nodules, pulmonary nodules, and central nervous systems nodules. These nodules occur almost exclusively in association with rheumatoid arthritis. Very rarely do rheumatoid nodules occur as rheumatoid nodulosis in the absence of rheumatoid arthritis. Rheumatoid nodules can also appear in other areas of the body other than the skin. Less commonly they occur in the lining of the lung or other internal organs. The occurrence of nodules in the lung of miners exposed to silica dust was known as Caplan’s syndrome. Rarely, the nodules occur at diverse sites on body.

Anti–citrullinated protein antibody Autoantibodies

Anti-citrullinated protein antibodies (ACPAs) are autoantibodies that are directed against peptides and proteins that are citrullinated. They are present in the majority of patients with rheumatoid arthritis. Clinically, cyclic citrullinated peptides (CCP) are frequently used to detect these antibodies in patient serum or plasma.

Lung nodule Medical condition

A lung nodule or pulmonary nodule is a relatively small focal density in the lung. A solitary pulmonary nodule (SPN) or coin lesion, is a mass in the lung smaller than three centimeters in diameter. A pulmonary micronodule has a diameter of less than three millimetres. There may also be multiple nodules.

Respiratory bronchiolitis Medical condition

Respiratory bronchiolitis is a lung disease associated with tobacco smoking. In pathology, it is defined by the presence of "smoker's macrophages". When manifesting significant clinical symptoms it is referred to as respiratory bronchiolitis interstitial lung disease (RB-ILD).

Tumor-like disorders of the lung pleura are a group of conditions that on initial radiological studies might be confused with malignant lesions. Radiologists must be aware of these conditions in order to avoid misdiagnosing patients. Examples of such lesions are: pleural plaques, thoracic splenosis, catamenial pneumothorax, pleural pseudotumor, diffuse pleural thickening, diffuse pulmonary lymphangiomatosis and Erdheim–Chester disease.

Asbestos-related diseases Medical condition

Asbestos-related diseases are disorders of the lung and pleura caused by the inhalation of asbestos fibres. Asbestos-related diseases include non-malignant disorders such as asbestosis, diffuse pleural thickening, pleural plaques, pleural effusion, rounded atelectasis and malignancies such as lung cancer and malignant mesothelioma.

References

  1. ↑ "Rheumatoid lung disease". Medline Plus Medical Encyclopedia.
  2. ↑ Chang-Miller, April. "Rheumatoid arthritis: Can it affect the lungs?". MayoClinic. Retrieved 20 February 2017.
  3. ↑ Nannini, Carlotta; Ryu, Jay; Matteson, Eric. "Lung Disease in Rheumatoid Arthritis". Medscape. Retrieved 20 February 2017.
  4. ↑ "Rheumatoid lung disease: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 2017-12-13.
  5. ↑ Lang, Florian (2009-03-19). Encyclopedia of Molecular Mechanisms of Disease. Springer Science & Business Media. ISBN   9783540671367.
  6. 1 2 3 4 Rangel-Moreno, Javier; Randall, Troy D. (2009). "Rheumatoid Lung Disease". Encyclopedia of Molecular Mechanisms of Disease. Springer, Berlin, Heidelberg. pp. 1857–1858. doi:10.1007/978-3-540-29676-8_3216. ISBN   978-3-540-67136-7.
  7. ↑ Stanislavsky, Alexandra. "Rheumatoid arthritis (pulmonary manifestations) | Radiology Reference Article | Radiopaedia.org". radiopaedia.org. Retrieved 2017-12-13.
  8. ↑ "Interstitial lung disease - Diagnosis and treatment - Mayo Clinic". www.mayoclinic.org. Retrieved 2017-12-13.
  9. ↑ Bongartz, Tim; Nannini, Carlotta; Medina-Velasquez, Yimy F.; Achenbach, Sara J.; Crowson, Cynthia S.; Ryu, Jay H.; Vassallo, Robert; Gabriel, Sherine E.; Matteson, Eric L. (June 2010). "Incidence and Mortality of Interstitial Lung Disease in Rheumatoid Arthritis: A Population Based Study". Arthritis and Rheumatism. 62 (6): 1583–1591. doi:10.1002/art.27405. ISSN   0004-3591. PMC   4028137 . PMID   20155830.
  10. ↑ MIALL WE, CAPLAN A, COCHRANE AL, KILPATRICK GS, OLDHAM PD. An epidemiological study of rheumatoid arthritis associated with characteristic chest x-ray appearances in coal-workers. Br Med J. 1953;2(4848):1231-1236. doi:10.1136/bmj.2.4848.1231
  11. ↑ "Round 16: Rheumatoid Lung Disease • Arthritis Information". Arthritis Information (in American English). Retrieved 2017-12-13.
  12. ↑ "Study Explores Risk Factors for RA-Associated Interstitial Lung Disease". MD Magazine. Retrieved 2017-12-13.
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