Diagnosis of sarcoidosis

During the last decade, many biochemical and immunologic advances have been made in the treatment and understanding of sarcoidosis. These studies have helped us to understand the basic mechanisms involved in granuloma formation, and many clinicians have used the information to diagnose and assess the activity of sarcoidosis. Further studies are needed to clearly establish the role of these advances in the everyday management of patients with sarcoidosis.     

T wave alternans in patients with cardiac sarcoidosis

There has been no report on T wave alternans (TWA) in patients with cardiac sarcoidosis. In this study, we investigated TWA in patients with cardiac sarcoidosis. Electrocardiographic recording for TWA was performed in 6 patients, using the CH2000 (Cambridge Heart). TWA was defined as positive in cases where alternans voltage of >1.9 μV and alternans ratio of >3.0 persisted more than 1 minute during exercise with ergometer with heart rate above 105 beats/min. TWA was positive in 3 patients, negative in 2 patients, and not determined in 1 patient. Arrhythmia was noted on Holter electrocardiogram in all 3 patients with positive TWA: sustained ventricular tachycardia in 1 patient and nonsustained ventricular tachycardia in 2 patients. Mean left ventricular ejection fraction in the 3 patients with positive TWA was 39.0%, whereas in the 2 patients with negative TWA it was 58.5%. A close relationship was suggested between TWA and severity of cardiac sarcoidosis.     

Radionuclide assessment of cardiac involvement in patients with sarcoidosis

This study was aimed to determine whether nuclear methods were useful for examining cardiac pathology and for making a decision of corticosteroid therapy in patients with sarcoidosis. Thirty six patients were divided into two groups; GpA consisted of 19 patients with cardiac sarcoidosis and abnormal ECG findings, and GpB of 17 patients with sarcoidosis without ECG abnormalities. Cardiac uptake of 67Ga-citrate in 2 and 99mTc-PYP in one of GpA was observed and steroid therapy resulted in the disappearance of the uptake. 201Tl-CL cardiac tomograms disclosed perfusion defects in 10 of 14 patients (71%) in GpA, including defects with redistribution in 8 of the 10 pts, but only one case in GpB. Radionuclide ventriculography using 99mTc-RBC revealed abnormal response of left ventricular (LV) function to exercise and LV dysfunction in GpA. These data suggest that nuclear study is a useful tool for diagnosing cardiac sarcoidosis, evaluating therapeutic effectiveness and long-term follow-up in patients with sarcoidosis.     

结节病诊治研究进展

结节病是一种病因不明的多器官受累的肉芽肿疾病,其临床表现多样,肺部和胸内淋巴结受累最为多见,容易导致误诊和漏诊.本文对结节病的诊治研究进展作一综述.     

Long-term follow-up CT scan evaluation in patients with pulmonary sarcoidosis

OBJECTIVES: The aim of the present study was to determine how the pattern and extent of sarcoidosis changes over time on serial high-resolution CT (HRCT) scans and to identify CT scan findings that might be helpful in predicting the prognosis of patients with the disease. METHODS: The initial and last HRCT scan findings of 40 patients with pulmonary sarcoidosis during a mean follow-up period of 7.4 years (range, 3 to 18 years) were evaluated retrospectively. HRCT scan findings then were correlated with the pulmonary function tests. RESULTS: Parenchymal abnormalities in most patients with a predominant nodular pattern (18 patients) and multiple large nodular pattern (8 patients) disappeared or decreased in size on long-term follow-up CT scans. A conglomeration pattern (five patients) shrank and evolved into bronchial distortion. The shrinkage of the conglomeration pattern correlated with a decline of FEV1/FVC ratio, despite an improvement in FVC. A ground-glass opacity pattern (five patients) and a consolidation pattern (three patients) evolved into honeycombing. The evolution of the ground-glass opacity and consolidation patterns into honeycombing occurred along with a decline in FVC, although the parenchymal abnormalities became smaller. CONCLUSION: Patients with a predominantly ground-glass opacity pattern and consolidation pattern seen on the initial CT scan had a worse prognosis and were susceptible to developing severe respiratory insufficiency. The predominant patterns seen on the initial HRCT scan may be helpful in predicting the outcomes of patients with sarcoidosis.     

Implantable cardioverter defibrillator therapy in patients with cardiac sarcoidosis

ICD Shocks in Cardiac Sarcoidosis . Background: An implantable cardioverter defibrillator (ICD) is indicated for some patients with cardiac sarcoidosis (CS) for prevention of sudden death. However, there are little data regarding the event rates of ICD therapies in these patients. We sought to identify the incidence and characteristics of ICD therapies in this patient population. Methods: We performed a cohort study of patients with ICDs at 3 institutions. Cases were those patients with CS and an ICD implanted for primary or secondary prevention of sudden death. Additionally, we included a comparison with historical controls of ICD therapy rates reported in clinical trials evaluating the ICD for primary and secondary prevention of sudden death. Results: Of the 112 CS subjects identified, 36 (32.1%) received appropriate therapies for ventricular tachyarrhythmias (VT) over a mean follow‐up period of 29.2 months. VT storm (>3 episodes in 24 hours) occurred in 16 (14.2%) CS subjects. Inappropriate therapies occurred in 13 CS subjects (11.6%). Covariates associated with appropriate ICD therapies included left ventricular ejection fraction (LVEF) <55% (OR 6.52 [95% CI 2.43–17.5]), right ventricular dysfunction (OR 6.73 [95% CI 2.69–16.8]), and symptomatic heart failure (OR 4.33 [95% CI 1.86–10.1]). Conclusions: In our cohort of patients with CS and ICDs, almost one‐third receive appropriate therapies. This may be due to a myocardial inflammatory process leading to increased triggered activity and subsequent scarring leading to reentrant tachyarrhythmias. Adjusted predictors of ICD therapies in this population include left or right ventricular dysfunction. (J Cardiovasc Electrophysiol, Vol. 23, pp. 925‐929, September 2012)     

Diagnosing isolated cardiac sarcoidosis

Abstract. Kandolin R, Lehtonen J, Graner M, Schildt J, Salmenkivi K, Kivistö SM, Kupari M (Helsinki University Central Hospital, Helsinki, Finland). Diagnosing isolated cardiac sarcoidosis. J Intern Med 2011; 270 : 461–468. Objectives. Cardiac sarcoidosis (CS) without clinically apparent extracardiac disease may escape detection because of the poor sensitivity of endomyocardial biopsy (EMB). We set out to analyse our experience of repeated and imaging‐guided biopsies in clinically isolated CS. Methods. We retrospectively reviewed the medical records, laboratory test results, imaging studies and pathological analyses of 74 patients with either histologically proven or clinically probable CS at our institution between January 2000 and December 2010. Results. Fifty‐two patients had histologically proven CS, of whom 33 (26 women) had disease that was clinically isolated to the heart. Sarcoidosis was detected in the first EMB in 10 of the 31 patients who underwent biopsy. CS was found by repeated EMBs, targeted by cardiac imaging, in seven additional patients, and 11 patients were diagnosed by sampling 18‐F‐fluorodeoxyglucose position emission tomography‐positive mediastinal lymph nodes at mediastinoscopy. Together, the first biopsy (cardiac or mediastinal lymph node) provided the diagnosis in 34%, the second biopsy in 31% and the third in 22% of biopsied patients with isolated CS. Four (13%) of the remaining diagnosis were made after cardiac transplantation and one in a patient who did not undergo biopsy) at autopsy after sudden cardiac death. Conclusions. Cardiac sarcoidosis may present without clinically apparent disease in other organs. At least two‐thirds of patients remain undiagnosed after a single EMB session. The detection rate can be improved by repeated and imaging‐guided cardiac or mediastinal lymph‐node biopsies. Nevertheless, false‐negative biopsy results remain a problem in CS patients with no apparent extracardiac disease.     

Diagnosis and treatment of hepatic sarcoidosis

Opinion statement The presence of granulomas in the liver raises consideration of a wide differential diagnosis, but in most Western series, sarcoidosis accounts for a majority of cases. This review will focus specifically on the diagnosis of and therapy for hepatic sarcoidosis. Sarcoidosis is a systemic granulomatous disease of unknown etiology. Hepatic involvement of sarcoidosis was described in 11.5% of 736 patients enrolled in the ACCESS study. However, presence alone of granulomas in an organ in sarcoidosis does not dictate treatment. The decision to treat should be based on symptoms and severity of disease. Although hepatic involvement usually is asymptomatic, a minority of patients progress to chronic cholestatic disease, portal hypertension, and cirrhosis that may require liver transplantation. Treatment of hepatic sarcoidosis should be reserved for patients who manifest this spectrum of disease. Glucocorticoid treatment is first-line therapy for hepatic sarcoidosis, improving symptoms and abnormal laboratory values but generally having no effect on progression of disease. In addition to glucocorticoids, immunomodulators such as azathioprine, methotrexate, hydroxychloroquine, and infliximab have been used with some positive effects on symptoms, liver enzyme abnormalities, and hepatomegaly, but none has been shown to prevent progression of disease. Ultimately, in cases of overt liver failure, liver transplantation is the definitive treatment. Overall, treatment for hepatic sarcoidosis is targeted toward alleviation of symptoms but has no curative potential at this time. Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure.     

Primary cardiac tumors: retrospective evaluation of 15 consecutive patients

During a decade (1975-1985) 15 patients have been treated for a primary cardiac tumor at the University Hospital of Ghent. Histological examination revealed both benign (n = 12) (myxoma, pseudomyxoma, myxoid degeneration) and malignant (n = 3) (angiosarcoma) tumors. Tumor diagnosis generally was made by echocardiographic and/or angiocardiographic examination of patients presenting with various constitutional and/or cardiac symptoms. In two patients however diagnosis became obvious by histological examination of the material removed during acute embolectomy. All tumors were surgically resected with the aid of extracorporeal circulation. Benign tumors have generally good prognoses, although rhythm or conduction disturbances, complete obstruction or embolization can entertain malignant consequences. Three of our patients died of such a complication. Therefore all cardiac tumors should be resected as soon as possible after diagnosis. One patient showed evidence of recurrence of the tumor. Malignant heart tumors have bad prognoses: all three of our patients died within two years.     

Closed mitral valvotomy: early results and long-term follow-up of 3724 consecutive patients

Between 1956 and 1980 closed mitral valvotomies were performed in 3724 consecutive patients (male:female ratio 1.1:1) with mitral stenoses. Their ages ranged from 6 to 69 years, with a mean (SD) of 27.3 (9.3). Mitral stenosis in the younger age group is a unique condition and a great majority of these patients rapidly develop significant pulmonary hypertension and congestive cardiac failure. In this study a large number of subjects belonged to functional class IV (41.5%). Hospital mortality was 1.5% over the last 5 years. After valvotomy, 11 patients (0.3%) developed severe mitral regurgitation that made valve replacement necessary in the immediate postoperative period. Early postoperative embolism occurred in 0.4% of those who were in atrial fibrillation and had preoperative anticoagulation whereas it occurred in 0.95% of those in sinus rhythm who had no anticoagulation. Late postoperative embolism occurred at a rate of 0.3 to 1.6 per 1000 patients per year over a 20 year period. Rheumatic reactivity occurred at a rate of 1.3 to 2.2 per 1000 patients per year during the same period. Rate of occurrence of restenosis varied from 4.2 to 11.4 per 1000 patients per year between the fifth and fifteenth year of follow-up. Closed transventricular revalvotomy for restenosis was accomplished in 130 subjects with a 6.7% mortality. Excellent symptomatic improvement was evident in 86% of long-term survivors at the end of 15 years. Actuarial survival was 95%, 93.1%, 89.5%, and 84.2% at 6, 12, 18, and 24 years, respectively, after closed mitral valvotomy.(ABSTRACT TRUNCATED AT 250 WORDS)