[Heart involvement in systemic sclerosis: analysis of four cases]. / Zajecie serca w twardzinie ukladowej na podstawie analizy czterech przypadków klinicznych.

INTRODUCTION: Systemic sclerosis (SSc) is an autoimmune disease characterized by multisystem involvement. Heart involvement occurs in 80-100% of cases and represents one of the more common risk factors of death. Myocardial fibrosis and vascular microangiopathy lead to arrhythmias and impaired contractility with secondary left and right heart failure. Pulmonary arterial hypertension in some patients with systemic sclerosis results in right heart failure and low output syndrome during the end-stage of the disease. The aim of the present study was to analyze four cases of systemic sclerosis with severe cardiovascular complications and a fatal outcome. CASE DESCRIPTIONS: Case 1: A 68-year-old female who probably suffered from limited SSc (lcSSc) and pulmonary arterial hypertension for more than 10 years was unsuccessfully treated with vasodilatators (sildenafil, iloprost). Case 2: A 52-year-old female with a 5-year history of diffuse cutaneous SSc (dcSSc), interstitial lung disease, and pulmonary arterial hypertension underwent aggressive therapy with immunosuppressive and antiproliferative (treprostinil) agents but without effect on the progression of the disease. Case 3: A 50-year-old male with dcSSc and coexistent cardiomyopathy with dominating symptoms of right heart failure was placed on high doses of immunosuppressants (i.v. cyclophosphamide) and had a cardioverter-defibrillator implanted as part of primary prevention. The patient died with symptoms of severe, therapy-resistant, two-sided heart failure. Case 4: A 58-year-old female with a 6-year history of dcSSc, interstitial lung disease, and heart involvement with severe ventricular arrhythmias and significant mitral and tricuspid insufficiency received intensive immunosuppressive therapy (i.v. cyclophosphamide) and underwent arrhythmia ablation. Sudden cardiac arrest in this patient was attributed to arrhythmia due to cardiomyopathy associated with systemic sclerosis. CONCLUSIONS: Lung and heart involvement is the most common reason for poor prognosis in systemic sclerosis. Arrhythmias, usually latent clinically, and right heart failure associated with cardiomyopathy or pulmonary hypertension are the main reasons of cardiac death in SSc patients. Severe and fatal cardiovascular complications occur more often in dcSSc, particularly during the first few years after diagnosis. Early detection of cardiovascular manifestations should be a priority in systemic sclerosis.

Gastrointestinal involvement in patients with systemic sclerosis.

INTRODUCTION: Gastrointestinal (GI) involvement is a serious complication of systemic sclerosis (SSc). OBJECTIVES: The aim of the study was to determine the incidence of GI manifestations in SSc. PATIENTS AND METHODS: We studied 73 patients with SSc (60 women and 13 men). Diffuse cutaneous SSc (dcSSc) was diagnosed in 30 patients and limited cutaneous SSc (lcSSc) in 43 patients. Upper GI involvement was assessed based on clinical symptoms such as dysphagia and gastroesophageal reflux-related complications. The majority of patients underwent radiographic examination including a barium swallow. Lower GI involvement was evaluated on the basis of such clinical symptoms as constipation and diarrhea. RESULTS: GI symptoms were observed in 54 (74%) SSc patients. Upper GI symptoms were observed in 54 (74%) patients and lower GI symptoms in 22 (30%) patients. The presence of anticentromere antibodies is associated with a lower risk of GI involvement. There are no significant differences in the incidence of pulmonary involvement between SSc patients with and without GI symptoms. CONCLUSIONS: GI involvement is observed in the majority of SSc patients. Clinical symptoms of GI involvement are significantly more common in patients with dcSSc. The incidence of upper GI symptoms is significantly higher than that of lower GI symptoms.

[Gout and comorbidities associated with hyperuricemia]. / Dna i schorzenia towarzyszace podwyzszonemu stizeniu kwasu moczowego.

Gout is a condition presenting with inflammatory arthritis caused by crystallization and phagocytosis of monosodium urate in synovial fluid. It is the most common form of arthritis in men above the age of 40 years. Four clinical stages of gout have been distinguished: asymptomatic hyperuricemia, acute gouty arthritis, intercritical gout, and chronic gout. Experimental and epidemiologic studies provide growing evidence that hyperuricemia is not only the driving force behind symptoms attributed to the deposition of monosodium urate in the musculoskeletal system but also the important etiological factor in common morbidities of modern societies such as arterial hypertension, cardiovascular disease, chronic kidney disease, and type 2 diabetes mellitus. Today, the majority of gout cases demonstrate clinical features of the metabolic syndrome. Recommendations of the European League Against Rheumatism (EULAR) published in 2006 address key issues in the diagnosis of gout, as well as in the nonpharmacologic and pharmacologic management with regard to the clinical condition and comorbidities of the individual patient. Routinely used antihyperuricemic drugs include allopurinol, colchicine, and uricosuric agents. New agents have recently been introduced into clinical practice, like pegylated uricase and febuxostat, a nonpurine inhibitor analog of xanthine oxidase. Thus, novel therapeutic options are now available to combat this chronic illness which often leads to significant disability.