Connective Tissue Diseases associated ILDs

Organs, bones and other parts of the body are connected by tissues that hold the body together. If these structures are harmed, it is called a connective tissue disease. Autoimmune diseases like rheumatoid arthritis, scleroderma and lupus belong to that group of diseases. Sometimes they occur together with interstitial lung disease. Connected tissue disease associated with ILD (CTD–ILD) represents a spectrum of diseases where the connective tissue of the body is inflamed. The most common amongst them are the following:
Systemic sclerosis (scleroderma)-associated ILD (SSc-ILD)
This rare disease is progressive in nature, meaning it might get worse over time. The symptoms of scleroderma can show in any part of the body, including the skin, muscles, blood vessels and internal organs. If the lung is affected by scleroderma, it could lead to fibrosis and inflammation in the walls of the air sacks of the lungs. The inflammation is caused by an overreaction of the body’s immune system, making it an autoimmune condition. When systemic sclerosis affects the lung, it most frequently leads to ILD or pulmonary hypertension, which slows down the blood flow through the lung. Most patients start suffering from scleroderma between the ages of 30 to 60. Women are four times more likely then men to develop the disease. Lung fibrosis affects most people with scleroderma to some extent, but only between 13 and 16 per cent of people will experience severe symptoms. Over time, one in ten people with severe scleroderma might develop serious lung symptoms that affect their everyday life.

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Symptoms:
Diagnosis and tests:
Treatment / management:
Symptoms:
Shortness of breath, fatigue, and non-productive cough
Diagnosis and tests:
High-resolution computed tomography scan of the chest
Treatment / management:
Medications, immunosupressants
Shortness of breath, fatigue, and non-productive cough
High-resolution computed tomography scan of the chest
Medications, immunosupressants
Rheumatoid arthritis-associated ILD (RA-ILD)
Rheumatoid arthritis is a long-term condition that causes pain, swelling and stiffness in the joints. The condition usually affects the hands, feet and wrists and is caused by a malfunction of the immune system. The body’s immune cells attack the tissue that surrounds the joints by mistake and damage joints, cartilage and nearby bone over time. This disease can also spread to the lung, in which case it presents as rheumatoid arthritis-associated ILD. As a serious pulmonary complication of rheumatoid arthritis, it is affecting 0.5 to one per cent of the population worldwide. Most patients with a diagnosis of rheumatoid arthritis are around 60 years old. Under the age of 75 years, women are four times more likely to develop rheumatoid arthritis then men.
Symptoms:
Diagnosis and tests:
Treatment / management:
Symptoms:
Shortness of breath, cough
Diagnosis and tests:
Physical examination, lung function tests, high-resolution computed tomography scan of the chest
Treatment / management:
Medications, immunosupressants
Shortness of breath, cough
Physical examination, lung function tests, high-resolution computed tomography scan of the chest
Medications, immunosupressants
Myositis-ILD
This disease causes inflammation or swelling of the muscle. This chronic condition often occurs together with other symptoms. There are several types of myositis, some that cause muscle weakness; others come with a skin rash. Early signs of the disease show in patients who have trouble getting up from a chair, climbing stairs, or grasping objects with their hands. Myositis–related autoimmune diseases such as polymyositis, dermatomyositis, amyopathic dermatomyositis, antisynthetase syndrome and mixed connective tissue disorder have an increased risk of developing into ILD.
Symptoms:
Muscle weakness, joint pain, skin rash, respiratory problems
Diagnosis and tests:
Hig-resolution computed tomography scan of the chest, muscle biopsy, lung function test
Treatment / management:
Combination of steroids and immunosuppressive medications, exercise training
Sjögren’s Syndrome
This long term auto-immune disease interferes with the ability of the body to produce moisture. Patients experience dryness in the mouth or the eyes, pain and fatigue. The disease can affect other organ systems including the lung, causing inflammation of the connective tissue. Interstitial lung disease (ILD) is considered the most frequent and serious pulmonary complication in Sjögren’s syndrome. Most patients are older than 40 when they get this diagnosis. For doctors it is challenging to recognize or diagnose because symptoms of Sjögren’s may mimic those of menopause, drug side effects, or medical conditions.
Symptoms:
Diagnosis and tests:
Symptoms:
Dry eyes, dry mouth, joint pain, skin rashes, persistent dry cough, prolonged fatigue
Diagnosis and tests:
Blood tests, eye tests, salivary flow test
Dry eyes, dry mouth, joint pain, skin rashes, persistent dry cough, prolonged fatigue
Blood tests, eye tests, salivary flow test