Systemic Sclerosis at an Intensive Care Unit: A Case Series and Literature Review.

INTRODUCTION: Systemic sclerosis (SSc) is a multisystemic autoimmune disease. Few studies have focused on the outcomes of SSC patients who require intensive care unit (ICU) admission, largely due to the absence of protocols for the optimal management of this disease during an ICU stay. OBJECTIVES: This study aimed to describe the outcomes of a series of SSc patients admitted to the ICU at a single center in Cali, Colombia. METHODS: Case series of SSc patients admitted to the ICU were reviewed. The main outcome was ICU mortality. Statistical analysis was performed with measures of central tendency and proportions. RESULTS: All the patients (n = 14) were female and either middle-aged or elderly; 9 (64%) were diagnosed with diffuse cutaneous sclerosis, and the remaining 5 patients with limited cutaneous sclerosis. Some were readmitted; therefore, the total number of ICU admissions was 21. The principal causes of ICU admissions were non-SSc-related causes (n = 15 [71.4%]). The respiratory system was the most involved on ICU admissions. The ICU mortality rate was 43% (n = 6). CONCLUSIONS: The severity of the disease at ICU admission and comorbidity are independently associated with ICU-related mortality. Furthermore, the optimal management of SSc patients includes accurate detection of SSc-associated organ involvement. More studies involving this category of patients are needed to establish the best effective protocols.

Stiff skin syndrome--case report.

Stiff skin syndrome is a rare scleroderma-like disorder of unknown etiology characterized by stone-hard indurations of skin, mild hypertrichosis and limited joint mobility. No effective treatment has yet been found. Exercises and rehabilitative therapy are important in maintaining the patient's quality of life. The authors present a case of a two-year-old boy with progressive skin hardening since he was eight-month old and secondary restricted joint mobility, diagnosed as Stiff skin syndrome.

Síndrome stiff skin: relato de caso / Stiff skin syndrome: case report

Síndrome stiff skin é doença rara, esclerodermiforme, de etiologia desconhecida, caracterizada por endurecimento pétreo da pele, hipertricose leve e limitação da mobilidade articular. Não há tratamento efetivo até o momento. Exercícios e reabilitação são importantes para manter a qualidade de vida do paciente. Os autores apresentam caso de um menino de dois anos de idade com endurecimento cutâneo progressivo desde os oito meses de idade e restrição secundária da mobilidade articular, diagnosticado como Síndrome stiff skin.

Stiff skin syndrome is a rare scleroderma-like disorder of unknown etiology characterized by stone-hard indurations of skin, mild hypertrichosis and limited joint mobility. No effective treatment has yet been found. Exercises and rehabilitative therapy are important in maintaining the patient's quality of life. The authors present a case of a two-year-old boy with progressive skin hardening since he was eightmonth old and secondary restricted joint mobility, diagnosed as Stiff skin syndrome.

Ciprofloxacin utility as antifibrotic in the skin of patients with scleroderma.

Scleroderma is an autoimmune connective tissue disorder that is characterized by microvascular injury, excessive fibrosis of the skin, and distinctive visceral changes that can involve the lungs, heart, kidneys and gastrointestinal tract. To date, although several drugs have been used to reduce fibrosis in scleroderma, there exists no effective pharmacological treatment. To determine if oral ciprofloxacin reduces the severity of scleroderma, a controlled, double-blind randomized clinical trial, with placebo, was conducted on 32 patients with diffuse and limited scleroderma, who received oral ciprofloxacin (250 mg) or placebo every 12 h. Skin induration and thickness of the patients were clinically evaluated using the modified Rodnan skin score at the beginning and once per month during 6 months of treatment with ciprofloxacin. To monitor progression of the disease, a monthly hematological exam and clinical evaluation was done to explore renal and hepatic function for each patient. Thirty patients completed the study; one from the treatment group was excluded when presenting a skin reaction and another from the placebo group abandoned the study due to an exacerbation of disease. At the sixth month of the study, the ciprofloxacin group of patients showed a diminution in the modified Rodnan skin score (58% vs 18%, P = 0.003), showing no significant alterations in the laboratory assays in either groups of patients. Our results suggest that oral administration of ciprofloxacin for 6 months reduces the severity of symptoms affecting the skin of patients with systemic scleroderma, and does so without important secondary effects.