Introduction
Rheumatic diseases are a group of immunologically mediated inflammatory disorders affecting multiple organs. Even though they are uncommon, practically all pediatric rheumatic illnesses can cause lung disorders. Juvenile Systemic Lupus Erythematosus (JSLE), Scleroderma (Systemic Sclerosis [SSc]), Juvenile Dermatomyositis (JDM), Mixed Connective Tissue Disease (MCTD), Granulomatosis with Polyangiitis, and Juvenile Idiopathic Arthritis (JIA) are systemic inflammatory diseases with the highest likelihood of pulmonary involvement.
Although pulmonary problems are frequently less common in children than adults, they can impair function and raise mortality. Despite being clinically asymptomatic, patients frequently have impaired lung function, highlighting the significance of rheumatologists referring for pulmonary function tests and imaging upon diagnosis. Using biological agents and antifibrotic medications for juvenile patients with rheumatologic disorders are just two new developments defining the best ways to treat lung disease.
How Are Rheumatic Diseases Different in Children?
Regarding prevalence, clinical presentation, disease history, joint involvement, laboratory results, emotional impact, diagnostic hurdles, treatment concerns, long-term effects, and hereditary variables, rheumatic diseases in children differ from those in adults. Giving children with rheumatic disorders the best care and support requires understanding these disparities. Musculoskeletal (MSK) complaints in children are common. Many pediatric rheumatic diseases have different phenotypes, outcome measures, investigations, and treatments distinct from adult rheumatic diseases. The term "Juvenile Idiopathic Arthritis" (JIA) refers to a heterogeneous group of different disease subtypes distinguished by the start of arthritis before age 16 and symptoms that last longer than six weeks after other causes of juvenile arthritis have been ruled out. The joint effusion or two or more of the following symptoms—limited range of motion, joint line soreness, painful range of motion, and warmth—are used to diagnose arthritis.
Polyarticular JIA, subdivided into rheumatoid factor positive and rheumatoid factor negative, accounts for 10 % – 30 % of JIA cases occurring most commonly in young girls with an early peak between ages 1 – 4 years and a later peak of 6 –12 years. Systemic onset JIA accounts for 10 % of cases of JIA with a broad peak of onset between 1 and 5 years, and it also occurs in adolescence and adulthood.
Children of both sexes are equally affected. Fever, exhaustion, and anemia are common systemic symptoms that might precede or eclipse arthritis by six weeks to six months. The arthritis is often polyarticular, symmetrical, and can be severe and treatment-resistant. The systemic manifestation includes fever spikes >38.5 °C occurring once or twice daily, which return to baseline or below temperatures.
What Are the Clinical Manifestations of Lung Involvement in Children With Newly Diagnosed Rheumatic Diseases?
Unique clinical symptoms in children not frequently seen in adults are possible. For instance, systemic symptoms and fever are frequently present in JIA in children but not in adult-onset rheumatoid arthritis. Rheumatic disorders are a wide range of inflammatory conditions that can harm a child's lungs, among other organs. Early diagnosis and effective care are essential since these illnesses frequently have complex clinical presentations. In this article, we will examine the clinical symptoms of lung involvement in children with newly identified rheumatic illnesses. The likelihood of developing lung problems increases in pediatric children with newly diagnosed rheumatic disorders who have not yet received treatment.
Additionally, a study found a correlation between a heightened risk of lung involvement and both CD4 and CD8 levels and an increase in Erythrocyte Sedimentation Rate (ESR). However, reports regarding the detection of lung involvement in children with treatment-naive, newly diagnosed rheumatic diseases are scarce. Patients with rheumatic diseases had abnormal HRCT (High-Resolution CT).
1. Systemic Lupus Erythematosus (SLE)
The lungs can be impacted by the systemic autoimmune disease known as SLE. Pleuritis, pneumonitis, and pulmonary hypertension are pulmonary symptoms in children with SLE. In contrast to pneumonitis, which can cause fever, dyspnea, cough, and bilateral infiltrates on chest imaging, pleuritis can cause chest discomfort, a cough, and pleural effusion. Respiratory distress, exhaustion, and exercise intolerance are potential effects of pulmonary hypertension. Rapid diagnosis and treatment are necessary because lung involvement can be severe and life-threatening.
2. Systemic Sclerosis
A rare form of connective tissue disease called systemic sclerosis is characterized by fibrosis in the skin and internal organs, including the lungs. The most frequent pulmonary symptoms of Interstitial Lung Disease are increasing dyspnea, a dry cough, and decreased lung function. Another serious complication of SSc is Pulmonary Arterial Hypertension (PAH), which causes exercise intolerance, syncope, and right heart failure. Effective management of pulmonary involvement in SSc requires close monitoring and early intervention.
3. Kawasaki Disease
Acute vasculitis, known as KD, typically affects young children. Although pulmonary involvement is uncommon, it can present as interstitial pneumonitis, myocarditis, and coronary artery aneurysms. Patients may exhibit respiratory discomfort and cough in severe situations. Preventing significant lung consequences from KD depends on prompt diagnosis and treatment.
4. Sjogren's Syndrome
SS is an autoimmune condition that causes the mucous membranes to dry but can also affect the lungs. The two most common pulmonary symptoms, Lymphocytic Interstitial Pneumonitis and Interstitial Lung Disease cause dyspnea, coughing, and exhaustion. Children with SS may fare better in the long run with early detection and effective care of lung involvement.
5. Juvenile Idiopathic Arthritis
JIA is the most prevalent rheumatic illness in kids, and lung involvement is an uncommon but severe consequence. Pleuropulmonary involvement, which causes pleuritis, pleural effusion, and occasionally interstitial lung disease, is the main pulmonary symptom of JIA. Children may have a dry cough, dyspnea, pleuritic rub, and chest pain. Pleural thickening, effusion, and reticular or nodular opacities are radiographic findings. Early detection and care are essential to stop lung injury progression.
Conclusion
Numerous pulmonary symptoms in children with newly identified rheumatic disorders can significantly negatively influence their prognosis and quality of life. Preventing irreparable lung damage and improving patient outcomes need early detection, careful examination, and proper management. Clinicians must look for indications of lung involvement in children with rheumatic disorders, work with multidisciplinary teams to provide interdisciplinary care, and customize treatment plans to meet each patient's needs. We can handle lung involvement in these complex illnesses more effectively due to ongoing research and developments in the field.
