Statin-Induced Necrotizing Autoimmune Myopathy: Case Report of a Patient under Chronic Treatment.

Introduction: 3-Hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR) inhibitors are widely used worldwide to treat dyslipidaemia and prevent cardiovascular events. Statins can cause a wide variety of muscle injuries ranging from myalgia to severe rhabdomyolysis. In most cases, these symptoms are mild and self-limiting and do not require specific treatment besides drug withdrawal. Statin-induced autoimmune necrotizing myopathy (SINAM) is a rare but potentially fatal complication, characterized by the subacute onset of progressive proximal muscle weakness and considerably high creatine phosphokinase (CK) levels in patients exposed to statins. The diagnosis is supported by the presence of antibodies HMGCR, which allows the differentiation from other forms of necrotizing autoimmune myopathies. Symptoms usually progress even after statin discontinuation and can determine severe muscle damage. Summary. We describe the case of a 77-year-old man who developed SINAM after 5 years of statin use. He suffered from muscle functional impairment mainly involving proximal lower limb muscles which progressed to the point that he almost became bedridden. Initial treatment with prednisone alone was not effective, and he required a combination therapy with steroids, methotrexate, and intravenous immunoglobulins. After 5 months of therapy and rehabilitation, he showed complete laboratory response and muscle strength recovery. Conclusion: Recognizing SINAM is paramount in order to promptly start treatment and avoid permanent muscle damage. Using a combination therapy from the beginning could contribute to a better outcome. Prompt statin cessation, categorization of the muscle disease by autoantibody testing, imaging, and histology, exclusion of malignancy, and anti-inflammatory therapy with corticosteroids, antimetabolites, immunoglobulins, and in some cases rituximab are currently accepted approaches to this entity.

Small intestinal bacterial overgrowth in patients suffering from scleroderma: clinical effectiveness of its eradication.

OBJECTIVES: After the skin, the gastrointestinal tract is the second most common target of systemic sclerosis (SSc). AIM: Our aims were to investigate orocecal transit time (OCTT) and the presence of small intestinal bacterial overgrowth (SIBO) in SSc as a cause of intestinal symptoms. METHODS: Fifty-five SSc patients and 60 healthy controls, sex and age matched, entered the study. Enrolled subjects completed a questionnaire for intestinal symptoms and a global symptomatic score (GSS) was calculated. OCTT and the presence of SIBO were assessed by a lactulose breath test (LBT). Patients with SIBO were treated with rifaximin 1,200 mg/day for 10 days. Finally, a second questionnaire and LBT were performed 1 month after the end of therapy. RESULTS: The prevalence of SIBO was higher in SSc patients compared with controls (30/54 vs 4/60, respectively, P < 0.001). OCTT was significantly slower in SSc patients compared with controls (150 min, 25-75th percentile 142.5-165 vs 105 min, 25-75th percentile 90-135, respectively, P < 0.001). In patients with SIBO, the median GSS score was 8 (25-75th percentile 3.25-10.75). Eradication of SIBO was achieved in 73.3% of patients, with a significant reduction of symptoms in 72.7% of them (GSS score 2, 25-75th percentile 1-3, P < 0.05). CONCLUSIONS: These data suggest that SIBO occurs more frequently in SSc patients than in controls. Intestinal symptoms in these patients may be related to this syndrome and its eradication seems useful to improve clinical features. OCTT is significantly delayed in SSc patients, suggesting an impairment of intestinal motility, a further risk factor for the development of SIBO.

Cardiac magnetic resonance imaging detects subclinical right ventricular impairment in systemic sclerosis.

OBJECTIVE: To assess myocardial involvement in patients with systemic sclerosis (SSc) with no signs or symptoms of cardiac impairment (New York Heart Association functional class I). METHODS: Fifty patients (45 women, 5 men, age 53.3 +/- 12.9 yrs) who did not complain of serious diseases other than SSc were recruited out of 119 consecutive patients with SSc. Thirty-three were found to have limited cutaneous SSc (lSSc) and 17 diffuse SSc (dSSc). All underwent cardiovascular magnetic resonance imaging (MRI) to determine right and left systolic and diastolic volumes and ventricular ejection fractions (RVEF and LVEF). Thirty-one healthy subjects matched for sex, age, and body surface area (BSA) were studied as controls. Diffusion lung capacity test (DLCO) and high resolution computed tomography were performed to evaluate lung involvement. RESULTS: Disease duration between patients with lSSc (14.1 +/- 11.4 yrs) and those with dSSc (6.9 +/-4.4yrs) was found to be significantly different (p < 0.003). lSSc patients were older than those with dSSc (54.8 +/- 13.7 yrs vs 50.4 +/- 9.9 yrs, respectively; p < 0.04). Anticentromere antibodies and Scl-70 were positive in 23 (46%) and 17 patients (34%). Except for the left and right systolic volumes, all unadjusted cardiac MRI measures were significantly reduced in SSc compared to the controls (p < 0.001 and p < 0.009). These differences persisted after adjustment for subjects' height and BSA. Raw RVEF data and RVEF data matched for height and BSA were significantly reduced in dSSc patients in comparison to lSSc (p < 0.03). CONCLUSION: Compromised RVF was found in patients with asymptomatic SSc. Unlike standard diagnostic techniques, cardiac MRI appears to be a rapid and noninvasive means of determining subclinical right myocardial involvement that is otherwise undetected in patients with SSc.