What is Collagen Vascular Disease Associated With Interstitial Lung?
Interstitial lung disease (ILD) is a term that describes various lung conditions due to inflammation and/or scarring of the lung tissue. It’s often caused by collagen vascular diseases, which are a variety of illnesses triggered by the immune system attacking the body’s own tissues that can affect the lungs in specific ways.
The types of collagen vascular diseases that commonly impact the lungs include rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, polymyositis, dermatomyositis, mixed connective tissue disease, and Sjogren’s syndrome. These disorders can affect the lungs to different degrees, and can occur in both the early and late stages of the disease, which can impact a patient’s future health. Detecting lung involvement is crucial for determining the proper treatment and understanding the likely outcome for the patient.
Collagen vascular disease can be linked to various types of interstitial lung diseases, like usual interstitial pneumonia, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, diffuse alveolar damage, and lymphocytic interstitial pneumonia.
This summary has looked at the cause, distribution, development, history, evaluation, treatment and prognosis of interstitial lung disease that is related to collagen vascular disease.
What Causes Collagen Vascular Disease Associated With Interstitial Lung?
Collagen vascular diseases are disorders related to the immune system. They can happen on their own (be idiopathic) or be caused by many factors, including genes, the environment, drugs, smoking, and sex hormones. The exact reasons behind these diseases, which affect the connecting tissues in the body, largely remain a mystery. However, it’s well-known that dysfunctional immunity plays a big part.
Rheumatoid arthritis, one such disorder, is thought to be triggered by an infection, with a protein called ‘tumor necrosis factor’ causing tissue damage. There are also various proteins called ‘cytokines’ that contribute to inflammation, or swelling. Another disease, systemic lupus erythematosus, is believed to be the result of a mix of genetic susceptibility and environmental triggers. This triggers inflammation by auto-antibodies depositing in specific tissues. Some viruses, as well as bacteria like Ebstein-Barr, Mycoplasma, and Borrelia, have been linked to causing systemic lupus erythematosus. Vascular injury (damage to blood vessels) is thought to start the disease process in scleroderma. The underlying factors causing inflammation in Sjogren’s syndrome, polymyositis, and dermatomyositis, are similar.
A fresh classification has been proposed for a certain type of disease relating to connective tissues that doesn’t fit neatly into the recognized categories. Put forth by the European Respiratory Society/American Thoracic Society Task Force, they’ve named it “Interstitial pneumonia with autoimmune features (IPAF).”
The criteria for this classification are:
- Evidence of inflammation in the lung tissue through a biopsy or high resolution CT scan, ruling out other possible causes.
- Not having a previously defined connective vascular disease.
- Showing at least one characteristic from at least two of the following categories: clinical (such as ulcers, swelling, or morning joint stiffness), serological (specific antibodies or proteins in the blood), or morphological (related to the structure of the lungs, unexplained fluid in the lung or heart coverings, or suggested lung vessel disease).
Risk Factors and Frequency for Collagen Vascular Disease Associated With Interstitial Lung
About 30% of patients first diagnosed with Interstitial Lung Disease (ILD) also have Collagen Vascular Disease. Unfortunately, we don’t know exactly how often Collagen Vascular Disease and ILD occur together. In patients who have collagen vascular disease, up to 90% will also develop lung problems. In certain cases, where a scan called high-resolution computed tomography (HRCT) was used, ILD is found:
- 70% to 90% of the time in people with systemic sclerosis
- 4% to 68% of the time in people with rheumatoid arthritis
- 20% to 85% of the time in people with mixed connective tissue disease
- 10% to 30% of the time in people with Sjogren’s syndrome
- Up to 30% in systemic lupus erythematosus
Because of these high numbers, most guidelines suggest ruling out connective tissue disease when a person is newly diagnosed with ILD.
Rheumatoid arthritis affects up to 2% of people and is more common among women, though men in their middle age are more likely to develop ILD tied to this condition. Cases peak between the ages of 25 and 50. About half of the patients experience health troubles beyond joints, with lung disease being the second most common cause of death. About 5% of patients suffer from a severe type ILD, while a rapid and deadly form of this disease occurs rarely.
Progressive system sclerosis, also known as scleroderma, is a disease affecting multiple body systems most commonly among women aged 45 to 64. Patients with scleroderma experience the highest disease-related death rate, usually because of lung-related blood pressure issues, which affect 10% to 16% of these patients.
Systemic lupus erythematosus, a complex disease affecting mostly women and non-whites, rarely results in ILD. However, when it does, the disease typically presents as a nonspecific type of ILD.
Polymyositis and dermatomyositis mainly affect women aged 40 to 50. Between 5% and 30% of these patients develop ILD, usually a nonspecific one or one linked with a condition known as cryptogenic organizing pneumonia. This may even signal the upcoming onset of clinical myositis.
Mixed connective tissue disease, which presents as a combination of the above diseases, mostly affects female patients and is most common during the second and third decades of life. Typical lung problems show up in up to 66% of patients; the most common abnormalities are characterized as a hazy area on a lung image.
Sjogren’s syndrome, a condition where the immune cells infiltrate certain body organs, mainly affects women in their 30s and 40s. It’s more common among those with primary Sjogren’s syndrome and can manifest in various forms.
Signs and Symptoms of Collagen Vascular Disease Associated With Interstitial Lung
Interstitial lung disease is a condition that often manifests with symptoms like difficulty breathing during exercise and a dry cough. Some patients might also experience joint and muscle pain, fatigue, or general discomfort, potentially indicating an underlying condition called collagen vascular disease. Past medical and family histories can hint at certain diagnoses. For instance, a history of asthma could imply a condition called eosinophilic granulomatosis with polyangiitis, while a family history could suggest a collagen vascular disease, which can be hereditary.
Medication history is also important to consider, as some lung conditions can be induced by certain drugs. Lifestyle factors like smoking cigarettes, drug use, and certain types of occupational exposure need to be evaluated, since smoking has been firmly associated with pulmonary fibrosis, a type of lung disease.
(age-related details):
- Typically, interstitial lung disease associated with connective vascular disease often affects younger people (between 20 to 40 years).
- In contrast, idiopathic pulmonary fibrosis usually presents in older individuals (over 60 years).
During a physical exam, doctors might hear abnormal lung sounds, like rales or crackles. On the skin, there might be thickening, tiny, widened blood vessels (telangiectasias), or certain types of rashes. Some symptoms, such as dryness or swelling in the mouth and eyes, could hint at Sjogren’s syndrome, another type of immune system disorder.
Testing for Collagen Vascular Disease Associated With Interstitial Lung
The process of identifying interstitial lung disease involves several steps. First, your doctor may conduct a lung function test that could show a reduced capacity in your lungs, implying you might struggle to take in oxygen and expel carbon dioxide efficiently. This test, however, is not definitive and just serves as a guide toward the severity of the disease and how you might respond to treatment.
Your doctor might also run a test that looks for specific antibodies, which could indicate certain types of the disease. A surgical lung biopsy remains the most accurate way to determine your specific type of interstitial lung disease.
Chest X-ray is often the first imaging technique doctors use when they suspect interstitial lung disease. But while X-rays might reveal signs of the disease, such as unusual patterns in the lungs, they are not enough by themselves for a reliable diagnosis.
High-resolution computed tomography, which is a type of detailed CT scan, can show different patterns that are characteristic of various types of interstitial lung diseases. For instance, it might show various unusual features in the lungs consistent with diseases like nonspecific interstitial pneumonia, usual interstitial pneumonia, or lymphocytic interstitial pneumonia. Depending on the disease, the lungs might display changes in the texture, like ground-glass opacities, or show structural changes, like honeycombing and thickening in specific areas.
As part of the procedure, the doctor may also check for signs of specific diseases, such as rheumatoid arthritis, systemic lupus erythematosus, polymyositis, dermatomyositis, mixed connective tissue disease, and Sjogren’s syndrome, all of which could be associated with different types of interstitial lung disease.
Remember, the exact imaging features can vary significantly, even within the same disease, and a combination of these factors would be used for a diagnosis. The extent of specific features, such as honeycombing, can also help your doctor measure the severity of the disease and how it might affect your breathing capacity.
Treatment Options for Collagen Vascular Disease Associated With Interstitial Lung
Treatment for interstitial lung disease, which is often associated with collagen vascular diseases, aims to manage the underlying connective tissue disease. Both drug and non-drug therapies are used. Drug treatments usually include immunosuppression with corticosteroids, while non-drug therapies involve oxygen support, physical exercise, and lung rehabilitation. The choice of treatment may differ based on the type of the lung disease as the response to treatment can vary.
Immunosuppression, which is the suppression of the body’s immune response, is the main treatment method. Before starting this type of treatment, patients are screened for infections and checked for organ dysfunction, since the treatment could increase the risk of certain infections.
For patients with lung diseases related to Sjögren syndrome, a connective tissue disease, Prednisone is usually the first treatment option, especially when symptoms are worsening. If Prednisone is not effective or causes side effects, other immunosuppressive drugs, such as mycophenolate or azathioprine may be used.
For patients with lung disease associated with rheumatoid arthritis, treatment with Prednisone is usually recommended, unless there is evidence of a lung infection. The effect of the treatment typically takes one to three months to show.
In cases of rapidly progressing lung disease associated with systemic lupus erythematosus, strong immunosuppressive treatments, such as cyclophosphamide or rituximab, are often needed to control inflammation and minimize lung damage.
Recent treatments for interstitial lung disease have also included antifibrotic medications, which can slow down the development of fibrous tissue in the lungs. This type of treatment has shown variable efficacy.
For patients with late-stage lung disease, lung transplantation can be considered. The survival rates within the first year of lung transplantation were reported to be similar for different associated diseases, including rheumatoid arthritis, IPF, and Scleroderma.
What else can Collagen Vascular Disease Associated With Interstitial Lung be?
When diagnosing interstitial lung diseases, doctors need to consider other causes. Conditions like sarcoidosis and occupational disorders that can cause damage in the area between the air sacs of the lungs might be behind the symptoms. Acute viral pneumonia, including lung infections due to COVID-19, might also have similar symptoms and appear the same on medical scans. Heart-related (Cardiogenic) and non-heart-related (non-cardiogenic) fluid buildup in the lungs can also lead to symptoms and changes on medical images that seem like they’re resulting from interstitial lung disease.
What to expect with Collagen Vascular Disease Associated With Interstitial Lung
Interstitial lung disease related to collagen vascular disease often leads to serious health issues and can be life-threatening. Still, this kind of interstitial lung disease usually carries a better survival outlook and tends to progress slower than idiopathic pulmonary fibrosis. The death rates are alike across all types of connective tissue disease-related forms of interstitial lung disease (including conditions like rheumatoid arthritis, scleroderma, and certain muscle and skin diseases). These rates have remained constant over the past 20 years.
The risk of death is highest in women, non-Hispanic white individuals, and those who develop high blood pressure in the lungs. Also at high risk are those who need more oxygen support and those needing mechanical ventilation. Nearly 20% of patients diagnosed with a nonspecific interstitial pneumonia, sadly, pass away within five years of their diagnosis.
The extent of the disease also matters. If a detailed lung scan shows more lung affected or the patient’s lung capacity is seriously reduced upon testing, these patients often face a higher risk of death.
Possible Complications When Diagnosed with Collagen Vascular Disease Associated With Interstitial Lung
In patients suffering from collagen vascular disease, interstitial lung disease and pulmonary arterial hypertension are the leading causes of death and health complications. Other common issues that these patients deal with include drug toxicity and infections.
Medications used to treat rheumatoid arthritis, like gold salts and penicillamine, can lead to diffuse alveolar damage or obliterative bronchiolitis, which are conditions that impact the lung’s functionality. Additionally, Methotrexate therapy has been linked to pneumonitis, a condition causing inflammation in the walls of the alveoli in the lungs.
Preventing Collagen Vascular Disease Associated With Interstitial Lung
If you or a family member have had a history of collagen vascular disease and you’re experiencing certain physical symptoms, it’s a good idea to see a specialist like a rheumatologist or a pulmonologist. They can check for signs of a related lung disease, known as interstitial lung disease, which is sometimes associated with collagen vascular disease. The check-up may include a physical examination, tests to measure how well your lungs are functioning, and medical imaging like an X-ray or CT scan.