Introduction
Autoantibodies and organ damage caused by the autoimmune system are two characteristics of the diverse autoimmune disorders known as connective tissue diseases (CTDs). Serological tests confirm a diagnosis and assess disease activity by comparing it to CTDs. Numerous CTDs impact the lungs, either directly or as a therapeutic consequence. Breathing system components such as airways, arteries, parenchyma, pleura, and breathing muscles may be involved. Each patient had unique clinical and radiologic symptoms. Death rates are significant in CTD patients with pulmonary hypertension.
What Is the Correlation Between Pulmonary Manifestations of Rare Connective Tissue Disorders?
Most pulmonary manifestations may occur before, concurrently with, or after the clinical manifestation of the Connective Tissue Disorders (CTD). The frequency of each pulmonary manifestation associated with each CTD.
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The screening of CTD patients for pulmonary consequences needs to be better understood. However, the following approach can be applied to all CTDs, with clinicians customizing it to their patients' needs and clinical circumstances.
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Some individuals present with respiratory symptoms, whereas others are asymptomatic but exhibit physiological (pulmonary function test [PFT]) or radiological (HRCT) abnormalities associated with ILD. Depending on the severity of the disease, as indicated by HRCT, a beneficial step-by-step approach could be adopted.
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Although drug-induced lung injury is a rare complication, it should be included in the differential diagnosis of lung injuries. The prevalent and major side effects of CTD-ILD drugs make it easier to monitor them and follow up with patients. When drug-induced lung injury is suspected, it is critical to examine both the drug's dose and the interval between its administration and the development of pulmonary symptoms. There is no single test that can confirm this diagnosis. In addition to other findings, withdrawing the problematic medicine is the most effective diagnostic step. Bronchoalveolar lavage (BAL) is necessary to rule out infections (bacterial, viral, fungal, and mycobacterial).
What Are the Pulmonary Manifestations in Various CTD?
Systemic Lupus
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SLE is distinguished by the presence of autoantibodies and an immune response that causes injury to numerous organ systems. More females are impacted than males.
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Pleural effusions and pleuritis are the most common pulmonary symptoms. Infections are common and frequently result in severe pulmonary complications.
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Lung involvement in patients with SLE necessitates a thorough examination for infection, particularly bacterial or viral infections. Immunocompromised hosts should also be vigilant for fungal, opportunistic, and tuberculosis infections.
Pleural Disease: Patients with pleuritic chest pain have the disorder. The chest pain is increased by deep breathing, movements, or position changes and can be palpated to identify the uncomfortable locations.
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The pleural effusions are usually on both sides, small to average in size, and ruptured.
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Normal glucose, pH, and lower lactate dehydrogenase levels are linked to pleural fluid in SLE. Nonsteroidal anti-inflammatory drugs (NSAIDs) are usually used to treat lung disease in people with SLE. Immunosuppressant drugs may be needed for more serious diseases.
DAH: Diffuse alveolar hemorrhage is a life-threatening SLE pulmonary symptom. DAH is rare, occurring in fewer than four percent of SLE15 hospital admissions. Hemoptysis (coughing up blood from the respiratory tract), cough, and dyspnea (difficulty breathing.) The bleeding may lead to anemia.
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Chest radiography typically reveals bilateral alveolar infiltrates, suggesting pulmonary edema or infection.
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The diagnosis can be laboratory bronchoalveolar lavage samples showing bloody fluid and hemosiderin-laden macrophages; a positive culture result may rule out infection.
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High-dose glucocorticoids, Cyclophosphamide, and supportive measures have been shown to dramatically reduce the death rate among patients.
Blood Clotting Disorder
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About one-third of SLE patients have blood clotting disorder due to the presence of antiphospholipid antibodies (aPL), which are frequent. This can lead to fetal loss and arterial thrombosis (a blood clot that forms within an artery).
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The risk of thrombosis is around six times higher in people with SLE plus aPL than in people without aPL.
Rheumatoid Arthritis (RA): An autoimmune illness causes chronic, persistent autoimmune conditions, mostly impacting the joints.
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The condition is twice as common in women and peaks between 40 and 60.
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The risk of pulmonary involvement is associated with smoking, male gender, severe joint disease, positive respiratory fluid, and extra-articular (outside the joint) symptoms.
Bronchiectasis: In early RA, patients experience bronchiectasis, including shortness of breath, cough, coughing up blood, and recurring infections.
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Pulmonary function tests generally reveal airway blockage.
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Long-term RA is associated with bronchiectasis, which can lead to persistent infections and respiratory insufficiency, resulting in death.
Pleural Disease: Pleural illness is frequent in RA patients but typically not symptomatic, as many patients may have asymptomatic pleural effusion while experiencing symptomatic effusion. It is more prevalent in men and often occurs with rheumatoid nodules and high-factor titers.
Interstitial Lung Disease: ILD is the most prevalent lung symptom, causing significant patient morbidity and mortality.
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In investigations utilizing chest high-resolution CT scanning, ILD screening in RA patients showed a prevalence; it is more prevalent in men.
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High rheumatoid factor titers are linked to ILD and reduced carbon monoxide diffusing capacity (DLCO).
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Dyspnea during exertion and nonproductive cough are prevalent. The physical exam may show dry crackles on the pulmonary auscultation. The diagnosis of ILD in RA involves clinical pulmonary symptoms, consistent PFTs, and characteristic radiographic abnormalities.
Systemic Scleroderma: Systemic sclerosis (SSc) is an autoimmune illness defined by endothelial dysfunction, small-vessel vasculopathy, excessive collagen synthesis, and fibrosis.
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Lung disease is prevalent in systemic sclerosis patients. Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the primary causes of death in the lungs.
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The main symptom of SSc is skin thickening, and the extent of cutaneous involvement determines its subgroups.
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Patients with limited cutaneous SSc often experience skin thickening on limbs below elbows and knees and, to a lesser extent, the face and neck.
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Diffuse cutaneous SSc is characterized by significant skin thickening, including alterations near the elbows, knees, or trunk.
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Patients with diffuse cutaneous SSc have more severe symptoms such as skin thickening, digital edema, and arthritis.
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Patients are at significant risk for early ILD progression and scleroderma renal crisis. SSc-specific autoantibodies showed unique lung disease symptoms.
Vascular Disease: Systemic sclerosis is the most common connective tissue disorder linked to PAH.
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Approximately one-third of PAH patients are asymptomatic, with dyspnea on exertion and fatigue being the most prevalent symptoms.
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SSc-PAH patients had superior survival rates in the present therapy era.
Primary Sjögren's: Sjögren's syndrome (SjS) is a progressive autoimmune illness that causes exocrine glands to produce lymphocytic infiltration, resulting in decreased glandular function and dryness.
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The salivary and lacrimal glands are typically damaged, causing symptoms such as dry eyes and mouth.
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Complications of SjS in the respiratory system include airway dryness, interstitial lung disease (ILD), the formation of thickened tissue in the lining of the lungs, the accumulation of fluid in the pleural cavity, and the development of non-Hodgkin lymphomas.
Airway Illness: The upper airway is frequently impacted, causing dryness in the nasal mucosa, mouth, and trachea (xerostomia).
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Short airway involvement and air trapping can be seen in PFTs.
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Lung tissue histopathology investigations in Sjögren's syndrome patients with severe obstructive lung disease show lymphocytic or follicular bronchiolitis.
Conclusion
Numerous pulmonary problems, such as ILD, bronchiolitis, bronchiectasis, pleuritis, and pulmonary hypertension, are brought on by CTDs. ILD is a frequent and dangerous type of pulmonary involvement that shows different patterns of fibrosis and inflammation on HRCT scans and in lung biopsy specimens. Due to their common autoimmune character, the numerous CTDs linked to ILD are frequently grouped, yet each particular CTD has distinct clinical manifestations and therapy strategies for ILD.
