Cardiopulmonary Manifestations of Systemic Sclerosis Without Scleroderma and Advances in Treatment. Case report

Abstract

Systemic Sclerosis whithout scleroderma is a rare form of Limited Systemic Sclerosis in witch these patients don’t have important cutaneous symptoms. Case presentation: a 52 years old patient, with no relevant background, 17 years ago started with clinical symptoms like Raynaud Syndrome, photosensitive and paresthesia in both hands, in physical exam presented fine crackles and Skin thickening of the fingers, it was suspicion of collagen disease, and laboratory exams confirm the diagnose of Systemic Sclerosis whithout Scleroderma, because the cardiopulmonary symptoms were relevant. She was given management with systemic steroids. Four years later she had a complication with interstitial lung disease like pneumonitis and Pulmonary arterial hypertension and was managed with Azatriopina and Sildenafil with satisfactory results. Six years later she started with Rheumatoid Arthritis symptoms and started the treatment with Rituximab that still has. These days she has good prognosis with mild pulmonary disfuntion that doesn’t modifies daily activity. Conclusion: the characterization of the Systemic Sclerosis is vital, actually with known diagnostic criteria that are more accurate to manage the patient in the individual necessities, but still a lot remains to be investigated.