Arrhythmias and Conduction Disturbances in Autoimmune Rheumatic Disorders.

Rhythm and conduction disturbances and sudden cardiac death are important manifestations of cardiac involvement in autoimmune rheumatic diseases (ARD), which have a serious impact on morbidity and mortality. While the underlying arrhythmogenic mechanisms are multifactorial, myocardial fibrosis plays a pivotal role. It accounts for a substantial portion of cardiac mortality and may manifest as atrial and ventricular arrhythmias, conduction system abnormalities, biventricular cardiac failure or sudden death. In patients with ARD, myocardial fibrosis is considered to be the hallmark of cardiac involvement as a result of inflammatory process or to coronary artery occlusive disease. Myocardial fibrosis constitutes the pathological substrates for reentrant circuits. The presence of supraventricular extra systoles, tachyarrhythmias, ventricular activity and conduction disturbances are not uncommon in patients with ARDs, more often in systemic lupus erythematosus, systemic sclerosis, rheumatoid arthritis, inflammatory muscle disorders and anti-neutrophil cytoplasm antibody-associated vasculitis. In this review, the type, the relative prevalence and the underlying mechanisms of rhythm and conduction disturbances in the emerging field of cardiorheumatology are provided.

Pseudo-infarction pattern in diffuse systemic sclerosis. Evaluation using cardiovascular magnetic resonance.

BACKGROUND: Diffuse systemic sclerosis (dSSc) is characterized by vascular lesions and fibrosis. Cardiac involvement, although silent, accounts for 36% of deaths. We hypothesized that cardiovascular magnetic resonance (CMR) can clarify the pathophysiology of Q waves in dSSc patients. PATIENTS-METHODS: 105 dSSc, aged 48±2years, with atypical symptoms and normal routine assessment, were evaluated by ECG and CMR using a 1.5 T system. Biventricular function was assessed by steady-state free-precession sequence (SSFP). To identify fibrosis, late gadolinium enhanced areas (LGE) were evaluated 15min after injection of 0.2mmol/kg gadolinium-DTPA and expressed as % of LV mass. RESULTS: Q waves in V1-V5 (Group A), II, III, AVF (Group B) and I, AVL, II, III, AVF, V1-V5 (Group C) were found in 25/105, 8/105 and 5/105 dSSc, respectively. In 25 dSSc with Q in V1-V6, patchy intramyocardial LGE was detected in 24/25 and involved 8±2% of LV mass. LGE involved the intraventricular septum (IVS) in 11/24 and the lateral wall (LAT) in 5/24 dSSc. Only in 1/25 dSSc, an anterior, transmural LGE, due to LAD occlusion, was identified. In 8 dSSc with Q in II, III, AVF, patchy intramyocardial LGE was detected in the inferior wall and involved 5±2% of LV mass. In 5 dSSc with Q in V1-V5, II, III, AVF, patchy intramyocardial LGE was detected in anterior and inferolateral wall and involved 9±2% of LV mass. CONCLUSION: CMR unveiled that the pattern of myocardial fibrosis in dSSc with Q waves is due to the systemic disease and not to CAD.

IgG4-related disease: a rheumatologist's perspective.

OBJECTIVES: Given that the clinical features of several IgG4-related diseases (IgG4-RD) can mimic those of autoimmune disorders, the aim of this study was to find possible distinguishing characteristics that would help us identify such cases from the pool of patients in a rheumatology clinic. METHODS: From our clinic's medical records, we identified patients who fulfilled the recently published diagnostic criteria for IgG4-RD. We recorded their presenting features, co-morbid conditions, laboratory, radiologic and histologic findings as well as their treatment and outcome. RESULTS: We identified 11 cases of IgG4-RD: 4 cases of IgG4-related autoimmune pancreatitis (AIP), 5 cases of IgG4-related retroperitoneal fibrosis (RPF)/ periaortitis, 2 cases of IgG4-related sialadenitis and one of IgG4-related interstitial nephritis. 5 out of the 11 patients had been diagnosed with an autoimmune disease, namely rheumatoid arthritis (RA), Sjogren's syndrome (SS) and antiphospholipid syndrome (APS). 3 out of 11 patients were subsequently diagnosed with neoplastic disorders. All patients with IgG4-related AIP had raised CRP levels at presentation. Presenting features of RPF/periaortitis patients were constitutional symptoms, abnormal renal function, hypertension and back pain. Patients with IgG4-related sialadenitis had clinical features mimicking SS. The majority of patients had a favourable response to steroids. CONCLUSIONS: We present common IgG4-RD presentations in the setting of a rheumatology clinic. Increased awareness may avoid delay in diagnosis.

Noncompaction cardiomyopathy and pregnancy: An alarming coexistence ending in a favourable outcome.

Noncompaction of the left ventricular myocardium has gained increasing recognition over the past 25 years. This rare disease is caused by the arrest of myocardial morphogenesis. The classical triad of complications are heart failure, arrhythmias, including sudden cardiac death, and systemic embolic events. There is a paucity of data regarding women with left ventricular noncompaction cardiomyopathy and pregnancy outcome. The first report of an uneventful vaginal delivery without deterioration of left ventircular noncompaction cardiomyopathy is presented.

Real-time three-dimensional echocardiography in evaluating Libman-Sacks vegetations.

Libman-Sacks endocarditis, characterized by sterile fibrofibrinous vegetations that have the potential to develop anywhere on the endocardial surface, was originally reported in 1924. The mitral valve is most commonly affected, followed by the aortic valve, whereas tricuspid and pulmonary valves are seldom involved. Libman-Sacks vegetations can be found in approximately 1 of 10 patients with systemic lupus erythematosus by transoesophageal echocardiography (TTE), and they are variably associated with lupus duration, disease activity, anticardiolipin antibodies, and antiphospholipid syndrome manifestations. The capability to perform real-time 3D (RT3D) imaging in the evaluation of Libman-Sacks vegetation size may strengthen the already established role of transthoracic echocardiogram and TTE. The exact estimation of vegetation size may influence therapeutic interventions. Therefore, we are trying to highlight the role of RT3D echocardiography in assessing vegetation size in a patient with Libman-Sacks endocarditis.

Cardiovascular magnetic resonance angiography for the detection of anomalous coronary arteries: a case report.

Coronary artery anomalies occur in approximately 0.3% to 0.8% of the population, and include morphological variants of origin, course, or termination. Detection of these types of anomalously originating coronary arteries is crucial for therapeutic intervention.

Anomalous origin of the left circumflex artery from the right coronary artery: a case report.

INTRODUCTION: Coronary artery anomalies are found in 0.6% to 1.55% of patients who undergo coronary angiography, and the increasing use of diagnostic coronary angiography is uncovering even more such abnormalities. We present a very unusual case of an anomalous origin of the left circumflex coronary artery (LCx) from the proximal right coronary artery (RCA). CASE PRESENTATION: We present a case of a 45-year-old-man with a recent history of a non ST elevation myocardial infarction. The coronary angiography reveals an ectopic left circumflex coronary artery from the right coronary artery. In this report we attempted to highlight the rarity of this coronary anatomy. CONCLUSION: Anomalous origins of the coronary artery are rare, but may cause myocardial ischemia and sudden death. Thus, their reliable identification is a matter of paramount importance possibly evaluating the effects of therapeutic intervention.

Chest CT findings in patients with inflammatory myopathy and Jo1 antibodies.

Thoracic high-resolution computed tomography scans (HRCT) of 17 patients with inflammatory muscle disorders (IMD) and positive Jo1 antibodies were retrospectively reviewed regarding presence, extension, and distribution of pathological findings. Abnormal findings were found in 14 (82.3%) patients. The predominant CT abnormality was ground glass attenuation, which was present in seven patients (41.1%), having a bilateral and diffuse distribution. In general, lesions tended to appear in the lower lobes and more specifically in the lung bases. Interlobular septal thickening was found in six patients (35.3%); it was seen in the upper and lower lobes with peripheral distribution and bilateral localization in five out of six patients. Bronchiectases, reticular opacities, and honeycombing were found in six patients (35.3%). Air space consolidation was seen in about 17% of the patients. Lung involvement is a frequent feature of IMD patients with positive Jo1 antibodies and its most common radiological pattern is that of nonspecific interstitial pneumonia.

Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study.

OBJECTIVE: To assess the prevalence and pattern of myocardial fibrosis as detected by delayed enhanced magnetic resonance imaging (DE-MRI) in patients with systemic sclerosis (SSc), and to evaluate a possible association between myocardial fibrosis and cardiac arrhythmias. METHODS: Forty-one patients with SSc underwent 24-hour Holter monitoring, Doppler echocardiography, and DE-MRI following gadolinium administration. RESULTS: Technically acceptable DE-MRIs were obtained in 36 patients with SSc. Enhancement on DE-MRI, consistent with myocardial fibrosis, was observed in 24 of these patients (66%), and it was invariably midwall with a linear pattern, mostly involving basal and midcavity segments of the left ventricle. The volume of enhancement (total volume percentage index [TVPI]) did not differ between patients with diffuse SSc and those with limited SSc (mean +/- SD 1.46 +/- 1.73% versus 1.44 +/- 1.77%; P = 0.98). Patients with a long duration (> or = 15 years) of Raynaud's phenomenon had a greater number of enhancing segments (mean +/- SD 6.55 +/- 4.93 versus 2.96 +/- 3.46; P = 0.017) and a greater TVPI (mean +/- SD 2.44 +/- 1.97% versus 1.02 +/- 1.43%; P = 0.02) than those with a duration of Raynaud's phenomenon <15 years. Nineteen patients with SSc (53%) had abnormal Holter study results. Compared with patients with normal Holter study results, those with abnormal results had a greater number of enhancing segments (mean +/- SD 5.4 +/- 4.8 versus 2.5 +/- 2.9; P < 0.05) and a greater TVPI (mean +/- SD 2.1 +/- 1.9% versus 0.8 +/- 1.2%; P < 0.05). CONCLUSION: DE-MRI can identify myocardial fibrosis in a significant percentage of patients with SSc and may be a useful noninvasive tool for determining cardiac involvement.

Determinants of pulmonary arterial hypertension in scleroderma.

OBJECTIVE: To define risk factors associated with pulmonary arterial hypertension (PAH) in a large cohort of patients with systemic sclerosis (SSc). METHODS: SSc patients undergoing screening for PAH by means of Doppler echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded systolic pulmonary artery pressure along with pulmonary function testing, clinical, and laboratory data. PAH was defined as right ventricular systolic pressure equal or greater than 40 mm Hg. RESULTS: Of 114 SSc patients with echocardiographic measurements, PAH was found in 33 (29%) patients. In a multiple logistic regression analysis, the presence of pulmonary fibrosis on thoracic computed tomography (OR 6.78, CI 1.54 to 29.9), forced vital capacity less than 80% predicted (OR 3.03, CI 1.1 to 8.35), and duration of Raynaud's phenomenon preceding the onset of skin changes for at least 3 years (OR 5.75, CI 1.9 to 17.41) were found to be independent predictors of PAH. Age, disease duration, disease subtype, or autoantibodies were not associated with PAH in our patients. CONCLUSIONS: The present analysis identified pulmonary fibrosis and Raynaud's phenomenon preceding SSc skin manifestations by at least 3 years as risk factors for PAH in our scleroderma cohort. Screening for PAH in these high-risk patients may detect PAH at an earlier stage and guide decisions on therapeutic interventions.

Severe gastrointestinal involvement in systemic sclerosis.

Gastrointestinal tract (GIT) is the most common organ system involved in systemic sclerosis (SSc). GIT involvement is mainly attributed to GIT dismobility and wide mouth diverticular. GIT involvement in SSc can be also severely debilitating and even life threatening. To our knowledge, the presence of gastrointestinal bleeding due to the presence of multiple peptic ulcers in scleroderma patients is not well described. In this case report, we describe a scleroderma patient with recurrent gastrointestinal bleeding due to multiple peptic ulcers, in which vagotomy, pyloroplasty, and cholocystectomy were performed and subcutaneous somatostatin was administered to discontinue the recurrent bleeding and stabilize her clinical condition.

Interstitial lung disease in a patient with antisynthetase syndrome and no myositis.

Interstitial lung disease in patients with antisynthetase syndrome and no evidence of myositis is rare and may precede other disease manifestations. We report a patient who initially presented with symptoms primarily related to lung involvement. The diagnosis of the antisynthetase syndrome without myositis was made many months later when he developed a characteristic hand rash (mechanic's hands), which was confirmed by positive antibodies to Jo-1. With treatment, both the hand rash and the interstitial lung disease improved. Antisynthetase syndrome should be considered in patients presenting with interstitial lung disease with no evidence of myositis. Appropriate laboratory testing with measurement of specific autoantibodies may help in the early diagnosis and treatment of the syndrome.

Scleroderma lung: initial forced vital capacity as predictor of pulmonary function decline.

OBJECTIVE: To determine the ability of initial forced vital capacity (FVC) of patients with scleroderma to predict subsequent pulmonary function deterioration. METHODS: Data on 78 patients with scleroderma were retrospectively collected and analyzed. FVC (percent predicted), diffusing capacity for carbon monoxide (percent predicted), and various clinical and laboratory parameters were recorded. Pulmonary function decline (outcome) was defined as at least a 15-point sustained decrease in FVC (percent predicted). Kaplan-Meier analyses were performed separately for 60 patients initially assessed within the first 3 years from disease onset (group A) and 16 patients whose FVC values in the fourth or fifth year from disease onset were ascribed as baseline measurements (group B). RESULTS: Based on baseline FVC, patients in each group were categorized into those with normal FVC (> or =80% predicted) and those with decreased FVC (<80% predicted). In group A, the percent-predicted FVC of 89% of patients with normal initial FVC and of 75% of patients with reduced baseline FVC did not decrease by > or =15 points at 5 years (log rank P = 0.04). Four patients with decreased baseline FVC developed respiratory failure (FVC <50% predicted) versus none with normal initial FVC. Analysis of group B showed no difference between patients with normal baseline FVC and those with decreased FVC in the ability to further predict pulmonary function decline (log rank P = 0.13). Clinical and laboratory parameters (age, male sex, baseline diffusion capacity, anti-topoisomerase I, or duration of Raynaud's phenomenon preceding skin manifestations) were not associated with pulmonary function decline. CONCLUSION: Measured within the first 3 years from disease onset, baseline FVC (percent predicted) may predict deterioration of pulmonary function in patients with scleroderma. Patients with normal pulmonary function at initial assessment are at low risk to develop considerable impairment of pulmonary function.