Respiratory compromise can occur in systemic sclerosis solely on the basis of restriction in chest wall expansion in the absence of intrinsic lung disease and that this may have serious consequences. Respiratory muscle weakness with hypercapnic respiratory failure in systemic sclerosis has rarely been described in the literature, but the prevalence and mechanism of this have not been well characterized. In one previously reported case, chest wall infiltration from systemic sclerosis resulted in hypercapnic respiratory failure; in another reported case, a patient with systemic sclerosis developed hypercapnic respiratory failure secondary to severe ventilatory muscle weakness from a presumed myopathy of the respiratory muscles. Shrinking lung syndrome usually manifest in dyspnea, decreased lung volume associated with elevated diaphragm. Shrinking lung syndrome is rare but must be considered in patients with autoimmune disease and dyspnea.
Scleroderma Lung Disease â Other Lung Complications in Systemic Sclerosis: Maria do Socorro Teixeira Moreira Almeida
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Last date updated on July, 2014