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Pulmonary manifestations in scleroderma: A review
Abstract
Background: Scleroderma is a chronic multisystem autoimmune disease of unknown aetiology. Scleroderma is characterized by widespread obliterative vasculopathy of small arteries and is associated with varying degrees of tissue fibrosis and multiple organ involvement. Pulmonary disease is an important component of SSc. It is estimated that 80% of patients with SSc have some evidence of pulmonary disease. Systemic sclerosis has the poorest prognosis amongst rheumatology diseases with the highest case-specific mortality of any of the autoimmune rheumatic diseases as well as causing major morbidity.
Objective: This article will review pathogenesis, diagnosis and management of pulmonary disease in scleroderma.
Data source: Literature review of relevant published literature from both Africa and the rest of the world.
Data synthesis: The pathogenesis of lung disease in scleroderma involves a variety of pathways, including immunological/inflammatory activation and vascular injury. The primary cytokines responsible for the disease are unknown but it is postulated that it involves a complex interplay between inflammatory, B lymphocyte antibody production, oxidative stress and fibrotic pathways. This leads to the activation of lung fibroblasts by inflammatory and fibrotic mediators. Lung fibroblasts play a central role in the deposition of excess intracellular matrix. This inflammatory response leads to fibrosis and occurs in the setting of vascular derangements. The most common symptoms are dry cough and dyspnea on exertion. The high morbidity and mortality seen in SSc is generally attributed to the two major pulmonary manifestations of the disease: interstitial pulmonary fibrosis, or interstitial lung disease, and pulmonary arterial hypertension. Exertional dyspnea and dry cough are the most common presenting symptoms in patients
with SSc who develop pulmonary involvement Algorithm of diagnostic procedures in these patients does not differ considerably from the procedures of any other interstitial lung disease. At the current time, cyclophosphamide remains the best studied therapeutic agent although alternatives are actively being evaluated. The pathogenesis of pulmonary disease in scleroderma is still an enigma and is being actively researched. This will advance our understanding of the disease and ability to care for these patients.
Conclusion: Pulmonary complications are common in SSc and are the leading causes of death. Careful evaluation by the clinician is warranted to detect the presence of an ILD and to select patients appropriately for consideration of therapy. It is a major clinical challenge largely due to the enigma of the disease pathology as well as limited therapeutic options available. This is compounded by the perceived lack of evidence for clinical effectiveness of those treatments that are currently in use. Clinical trials are underway and offer hope for novel approaches to this mysterious and often devastating manifestation of scleroderma.
Objective: This article will review pathogenesis, diagnosis and management of pulmonary disease in scleroderma.
Data source: Literature review of relevant published literature from both Africa and the rest of the world.
Data synthesis: The pathogenesis of lung disease in scleroderma involves a variety of pathways, including immunological/inflammatory activation and vascular injury. The primary cytokines responsible for the disease are unknown but it is postulated that it involves a complex interplay between inflammatory, B lymphocyte antibody production, oxidative stress and fibrotic pathways. This leads to the activation of lung fibroblasts by inflammatory and fibrotic mediators. Lung fibroblasts play a central role in the deposition of excess intracellular matrix. This inflammatory response leads to fibrosis and occurs in the setting of vascular derangements. The most common symptoms are dry cough and dyspnea on exertion. The high morbidity and mortality seen in SSc is generally attributed to the two major pulmonary manifestations of the disease: interstitial pulmonary fibrosis, or interstitial lung disease, and pulmonary arterial hypertension. Exertional dyspnea and dry cough are the most common presenting symptoms in patients
with SSc who develop pulmonary involvement Algorithm of diagnostic procedures in these patients does not differ considerably from the procedures of any other interstitial lung disease. At the current time, cyclophosphamide remains the best studied therapeutic agent although alternatives are actively being evaluated. The pathogenesis of pulmonary disease in scleroderma is still an enigma and is being actively researched. This will advance our understanding of the disease and ability to care for these patients.
Conclusion: Pulmonary complications are common in SSc and are the leading causes of death. Careful evaluation by the clinician is warranted to detect the presence of an ILD and to select patients appropriately for consideration of therapy. It is a major clinical challenge largely due to the enigma of the disease pathology as well as limited therapeutic options available. This is compounded by the perceived lack of evidence for clinical effectiveness of those treatments that are currently in use. Clinical trials are underway and offer hope for novel approaches to this mysterious and often devastating manifestation of scleroderma.
