Woman with dyspnea.
Scleroderma is a disease of the overproduction of collagen fibers. The disease involves multiple organs with the lungs being the fourth most common. The most common organs are, in order, the skin, arteries, and the esophagus. Up to 90% of patients will present with history of Raynaud's phenomenon. Up to 60% present with the complaint of dyspnea. Usually the disease is diagnosed between 30-50 years of age and has 1:3 male to female ratio. The disease is rare, affecting 1.2/100,000.
To diagnose scleroderma, the American College of Rheumatology requires 1 major or 2 minor criterion. The major criteria is involvement of the skin proximal to the metacarpophalangeal joint. Minor criteria includes sclerodactyly, pitting scars, loss of finger tip tufts, bilateral pulmonary basal fibrosis. The prognosis is poor as the disease is progressive and indolent. The 5 year survival rate is 70%.
The best imaging clue is the dilatation of the esophagus with bilateral basilar interstitial thickening. Associated findings include low lung volumes due to restrictive lung disease, absorption of distal phalanges, phalangeal tuft calcifications.