Sine Syndrome in Systemic Sclerosis
The diagnosis of systemic sclerosis without scleroderma requires a careful medical history analysis and search for organ manifestations. Particularly in undifferentiated connective tissue diseases, severe organ changes can occur (heart, lungs, kidneys), b
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ntents Information on ssSSc from Clinical Practice ............................................................................. Summary of Presented Cases ..................................................................................................... Original Case Report: A Female Patient with Interstitial Pulmonary Involvement ................... Conclusion ................................................................................................................................. References ..................................................................................................................................
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Abstract
The diagnosis of systemic sclerosis without scleroderma requires a careful medical history analysis and search for organ manifestations. Particularly in undifferentiated connective tissue diseases, severe organ changes can occur (heart, lungs, kidneys), but skin involvement does not have to be present or occurs later. The analysis of anti-RNA polymerase III antibodies can help demarcate this rare subgroup of systemic sclerosis.
Systemic sclerosis (SSc) is a rare, chronic, autoimmune disease of unknown etiology. It is characterized by diffuse fibrosis, degenerative changes and vascular abnormalities that affect skin, joints and internal organs (especially the esophagus, gastrointestinal tract, lungs, heart and kidneys). In 1954 Abrams et al. [1], and in J. Rovenský (*) National Institute of Rheumatic Diseases, Nábrežie I. Krasku 4, Piešťany 921 12, Slovakia Institute of Physiotherapy, Balneology and Therapeutic Rehabilitation, University of Saint Cyril and Methodius, Trnava, Piešťany, Slovakia e-mail: [email protected] M. Vašáková Department of Respiratory Medicine, Thomayer University Hospital with Polyclinic, First Faculty of Medicine, Charles University, Prague, Czech Republic J. Rovensky et al. (eds.), Sine Syndromes in Rheumatology, DOI 10.1007/978-3-7091-1541-1_4, © Springer-Verlag Wien 2014
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J. Rovenský and M. Vašáková
1962 Rodnan and Fennel [2], published the first cases of systemic sclerosis without the presence of scleroderma (ssSSc – systemic sclerosis sine scleroderma). They described four patients: a 51-year-old male and three females, aged 59, 69 and 70, who died of systemic sclerosis with the absence of cutaneous changes. Later on, other cases were described [3–6].
Information on ssSSc from Clinical Practice Molina et al. [7] described the case of a patient with systemic sclerosis without scleroderma with renal crisis; organ failure was not associated with skin involvement. Symmetrical polyarthritis occurred 6 months later, followed by rapidly progressing renal failure without cutaneous changes. The diagnosis of renal sclerodermic crisis was confirmed histologically. Systemic sclerosis sine scleroderma was suspected and confirmed histologically, with the occurrence of typical cutaneous changes at a later period. Despite aggressive antihypertensive therapy that included ACE inhibitors and hemodialysis, her disease progressed to ends
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