Prognostic value of fragmented QRS in cardiac AL amyloidosis

Background

In light-chain (AL) cardiac amyloidosis, the 12-lead electrocardiogram (ECG) reflects myocardial amyloid infiltration with low limb voltages, pseudoinfarction patterns, and conduction abnormalities. Moreover, it is not unusual to see “aspecific” QRS complex abnormalities, such as notches and RsR′ pattern in the absence of QRS prolongation, i.e. a fragmentation of QRS complexes (fQRS), that has been associated with myocardial scars and prognosis. Since cardiomyocyte damage and interstitial fibrosis are associated with cardiac amyloid deposition, aim of the present study was to analyze the prevalence and the potential prognostic value of fQRS in patients with cardiac amyloidosis.

Methods

We enrolled 375 consecutive untreated patients in whom a first AL amyloidosis diagnosis was concluded between 2008 and 2010, 264 with and 111 without heart involvement. Patients with a positive history of coronary disease were excluded from the analysis.

Results

The prevalence of fQRS was significantly higher in patients with cardiac AL amyloidosis (28.5% vs. 11.7%; p = 0.0008). After a median follow-up of 561 days, Kaplan–Meier survival analysis revealed a significantly higher mortality in the fQRS group when compared with the “normal” QRS group (p = 0.0008). No association was found between the presence of fQRS and the duration of PQ, QRS, and QTc intervals, the presence of peripheral low voltages or pseudonecrosis, NT-proBNP serum levels or cardiac wall thickness.

Conclusions

In patients with cardiac AL amyloidosis, the presence of fQRS at diagnosis has an independent prognostic value. Such a simple and cheap analysis in patients' diagnostic work-up may improve diagnosis and prognostic stratification.     

Intérêt de l’imagerie par résonance magnétique nucléaire au cours de l’atteinte cardiaque du syndrome de Churg-Strauss. Trois observations et revue de la littérature

Purpose

The reported prevalence of cardiac complications is variable in patients with Churg-Strauss syndrome (15–92%) and depends on diagnostic tools. Diagnosis at early stage of heart involvement is crucial, resulting in appropriate management.

Methods

We report three patients who developed cardiac manifestations, revealing Churg-Strauss syndrome. The diagnosis of cardiac involvement was obtained using cardiac magnetic resonance imaging (MRI).

Results

Two patients were males and the remaining one was a female. Presenting clinical manifestations were: cardiac failure (n = 1) and retrosternal pain (n = 2). Laboratory findings disclosed: high blood count of eosinophils (range: 6000–11 000/mm³); antineutrophil cytoplasmic antibodies were positive in a single patient. Cardiac MRI demonstrated: (1) late gadolinium enhancement (n = 3), involving mainly the apical and mid-cavity left ventricular segments; (2) impaired left ventricular function (n = 2), mean left ventricular ejection fraction being: 51%; and (3) pericardial effusion (n = 3). Outcome was favourable after institution of combined therapy with prednisone and cyclophosphamide (n = 2); one patient also underwent plasma exchanges.

Conclusion

Our case series underlines that MRI is a helpful tool in the diagnosis of Churg-Strauss syndrome-related cardiac complications. We further suggest that clinical assessment of patients with Churg-Strauss syndrome should include cardiac MRI, in order to detect cardiac involvement at an early stage; indeed, because cardiac manifestations are predictive factors of poor prognosis, diagnosis at early stages of cardiac involvement may result in improvement of patients management.     

Prognosis Assessment of Cardiac Involvement in Systemic AL Amyloidosis by Magnetic Resonance Imaging

Background

Cardiac involvement is one of the most important prognostic factors in systemic AL amyloidosis. The aim of our study was to assess the role of cardiovascular magnetic resonance (CMR) imaging in prognosis evaluation in AL amyloidosis.

Methods

We retrospectively analyzed 29 consecutive patients with AL amyloidosis who had undergone CMR. Clinical, laboratory, echocardiographic, and CMR characteristics were compared between CMR-positive (ie, with CMR signs of cardiac localization of AL amyloidosis) and CMR-negative patients. Univariate and multivariate analyses were performed to assess the prognostic value of positive CMR in comparison with other prognostic factors.

Results

CMR was positive in 11 patients (38%). The overall survival rates for CMR-positive patients were 28%, 14%, and 14% versus 84%, 77%, and 45% at 1, 2, and 5 years, respectively, for CMR-negative patients (P = .002). Late gadolinium enhancement patterns, biventricular hypertrophy, and pericardial effusion on CMR were more frequent in nonsurvivors. Congestive heart failure, abnormal echocardiography, Eastern Cooperative Oncology Group grade >1, brain natriuretic peptide, and left ventricular ejection fraction <55% also were associated with a decreased survival. The presence of congestive heart failure was the only significant variable associated with survival on multivariate analysis.

Conclusion

We found that the presence of a positive CMR in AL amyloidosis was associated with a significantly increased risk of death, in particular of cardiac origin, but was not independent of clinical congestive heart failure.     

Valeur prédictive comparée de mortalité hospitalière du peptide natriurétique de type B et de l’albuminémie chez le sujet âgé hospitalisé pour insuffisance cardiaque aiguë sévère

Objective

To address the clinical relevance of serum albumin and B-type natriuretic peptide (BNP) concentration in the prediction of in-hospital death in elderly patients with acute severe heart failure.

Patients and methods

Seventy-four consecutive patients >70 years of age admitted for acute heart failure in NYHA class IV were prospectively included. BNP concentration was measured on admission and serum albumin concentration after clinical stabilization.

Results

Mean age was 86.6 ± 5.7 years. Sixty-five percent of patients had a normal left ventricular ejection fraction. Eighteen patients died during the in-hospital stay. Those patients who died were older, had higher blood urea nitrogen and BNP concentration, had lower systolic blood pressure and serum albumin concentration than patients who survived. Heart rate, rhythm, left ventricular ejection fraction, serum creatinine and hemoglobin did not differ according to outcome. By multivariate analysis, albumin (p = 0.0017), BNP (p = 0.016) and age (p = 0.03) were independent predictors of in-hospital death. Serum troponin I measured on admission in 71 patients was predictive of in-hospital death (p = 0.01), as well as serum total cholesterol measured after stabilization in 66 patients (p = 0.004). However, these two variables no longer predicted outcome in multivariate models, unlike serum albumin and BNP.

Conclusion

Serum albumin and BNP offer independent, additional information for the prediction of in-hospital death in elderly patient with acute severe heart failure regardless of left ventricular ejection fraction.     

Prevalence of acute cardiac disorders in patients with suspected ST-segment elevation myocardial infarction and non-significant coronary artery disease

Background and purpose

We aimed to study the prevalence of acute cardiac disorders in patients with suspected ST-segment elevation myocardial infarction (STEMI) and non-significant coronary artery disease (CAD).

Methods

From January to October 2012 we consecutively included patients admitted with suspected STEMI and non-significant CAD (coronary artery stenosis diameter < 50%). Patients were diagnosed with acute cardiac disorder in the presence of elevated cardiac biomarkers (troponin T > 50 ng/l or creatine kinase MB > 4 μg/l) or dynamic ECG changes (ST-segment changes or T-wave inversion).

Results

Of the 871 patients admitted with suspected STEMI, 11% (n = 95) had non-significant CAD. Of these, 67% (n = 64) had elevated cardiac biomarkers or dynamic ECG changes and were accordingly diagnosed with acute cardiac disorders. In the remaining 33% (n = 31) of patients, cardiac biomarkers were normal and ECG changes remained stationary.

Conclusions

Acute cardiac disorders were diagnosed in two thirds of patients with suspected STEMI and non-significant CAD.     

Anévrismes infectieux de l’aorte. À propos d’une série de dix patients

Purpose

Infectious aortic aneurysms are rare, being responsible of less than 3% of aortic aneurysms. In this study, we report the clinical characteristics and the outcome of patients presenting with infectious aortic aneurysms in an internal medicine unit.

Methods

Diagnosis of infection-related aortic aneurysm was obtained using: computed tomographic scan; and blood cultures, cultures and molecular biology testing of aortic wall and intra-aneurismal thrombus.

Results

The 10 consecutive patients included in this study consisted in eight men and two women with a mean age of 61.7 years. Patients presented with fever (n = 9), asthenia (n = 2), abdominal (n = 4) or chest pain (n = 1), lumbar pain (n = 3). Computed tomographic scan showed aneurysm involving both thoracic and abdominal aorta (n = 1), abdominal (n = 8) or thoracic aorta (n = 1). Isolated microorganisms were: positive Gram cocci (70%) and negative Gram bacilli (30%). All patients underwent both medical and surgical therapy. Outcome was favorable in nine patients; the remaining patient died from aneurismal aortic rupture.

Conclusion

Clinical manifestations revealing infectious aortic aneurysms are variable, including aneurysm rupture as well as atypical abdominal pain with inflammatory syndrome. These latter presentations are more common in patients, who are hospitalized in internal medicine. Our study underlines that this clinical pattern should not be ignored, in order to avoid both diagnostic and therapeutic delay that could lead to life-threatening complications and poor prognosis.     

Anomalies cardiaques après tentative de pendaison : étude descriptive préliminaire

Purpose of the study

Cardiomyopathy has sometimes been reported after suicide attempts by hanging. The objective of this retrospective observational study was to describe cardiac dysfunction occurring after hanging and its consequences on prognosis.

Patients and methods

Fifteen patients admitted to the intensive care unit for hanging from 1997 to 2011 were included and divided into two groups according to presence or absence of cardiac arrest at initial presentation. Cardiac dysfunction was defined by the presence of clinical, biological, electrocardiographic or echocardiographic abnormalities.

Results

Cardiac impairment was diagnosed in nine patients over 15 (60%). Of the six patients with initial cardiac arrest, only one survived without severe neurological sequellae. Among the nine patients without cardiac arrest, eight survived and five patients (56%) had cardiac impairment, including two cases of echocardiographic aspect of Takotsubo complicated by pulmonary edema. Mortality in intensive care was significantly related to the severity of the initial neurological state assessed by the Glasgow Coma Score (OR = 1.7; P = 0.02), and the occurrence of cardiac arrest (OR = 40; P = 0.016). The presence of cardiac involvement, reversible after the acute phase in all surviving patients was not associated with increased mortality.

Conclusion

In the aftermath of hanging, predictors of mortality are the presence of impaired consciousness or initial cardiac arrest, but not the occurrence of cardiac disease.     

Maze procedure in patients with left ventricular dysfunction

Background

The risks and benefits of a concomitant Maze procedure for patients with LV dysfunction undergoing major cardiac surgery have not yet been elucidated. This study aimed to evaluate the clinical impacts of the Maze procedure in patients with atrial fibrillation and left ventricular (LV) dysfunction.

Methods

Between January 1999 and March 2011, a total of 139 patients (mean age 52.7 ± 12.3 years, 54 females) with valvular atrial fibrillation (AF) and an LV ejection fraction (EF) of 40% or less underwent open heart surgery with (n = 77) or without (n = 62) a concomitant Maze procedure. We compared adverse outcomes (death and composite of death, thromboembolic events and congestive heart failure [CHF]) during a median follow-up period of 66.0 months (inter-quartile range, 27.5–106.9 months).

Results

Adverse events occurred in 41 patients, including 36 deaths, seven thromboembolic events and eight hospitalizations due to CHF. After adjustment for baseline profiles with the use of propensity scores and inverse probability weighting, patients who had the Maze procedure were at lower risks of death (hazard ratio, 0.39; 95% confidence interval, 0.16–0.93; P = 0.033) and composite adverse outcomes (hazard ratio, 0.28; 95% confidence interval, 0.14–0.57; P = 0.017) than those not undergoing the Maze procedure. Furthermore, the Maze procedure resulted in superior functional status (P < 0.001) and reduced the need for long-term anticoagulation therapy (67.1% vs. 91.2%, P = 0.001).

Conclusion

Performing the Maze procedure on patients with valvular AF and LV dysfunction reduced serious adverse outcomes and the need for long-term anticoagulation therapy when compared to cardiac surgery alone without the Maze procedure.     

AXL receptor tyrosine kinase is increased in patients with heart failure

Background

AXL is a membrane receptor tyrosine kinase highly expressed in the heart and has a conspicuous role in cardiovascular physiology. The role of AXL in heart failure (HF) has not been previously addressed.

Methods and results

AXL protein was enhanced 6-fold in myocardial biopsies of end-stage HF patients undergoing heart transplantation compared to controls from heart donors (P < 0.0001). Next, we performed a transversal study of patients with chronic HF (n = 192) and a group of controls with no HF (n = 67). sAXL and BNP circulating levels were quantified and clinical and demographic data were collected.sAXL levels in serum were higher in HF (86.3 ± 2.0 ng/mL) than in controls (67.8 ± 2.0 ng/mL; P < 0.0001). Also, sAXL correlated with several parameters associated with worse prognosis in HF. Linear regression analysis indicated that serum creatinine, systolic blood pressure and atrial fibrillation, but not BNP levels, were predictive of sAXL levels. Cox regression analysis indicated that high sAXL values at enrollment time were related to the major HF events (all-cause mortality, heart transplantation and HF hospitalizations) at one year follow-up (P < 0.001), adding predictive value to high BNP levels.

Conclusions

Myocardial expression and serum concentration of AXL is elevated in HF patients compared to controls. Furthermore, peripheral sAXL correlates with parameters associated with the progression of HF and with HF events at short term follow-up. All together these results suggest that sAXL could belong to a new molecular pathway involved in myocardial damage in HF, independent from BNP.     

Sclérites,aspects cliniques, étiologiques et thérapeutiques : à propos d’une série de 32 observations

Purpose

To report on the various clinical presentations, etiological diagnosis, prognosis and treatment of patients with scleritis evaluated at a tertiary care eye center.

Methods

Retrospective, monocentric study on a series of 32 patients in a tertiary center.

Results

The mean age of included patients with scleritis was 46.8 years (range, 22 to 77 years). Nineteen patients were women and 13 were men. Twenty-six patients (81%) had anterior scleritis (15 nodular, 8 diffuse and 3 necrotizing), six (19%) had posterior scleritis. Unilateral inflammation was present in 24 patients (75%). Twelve out of the 32 patients (37.5%) had an underlying systemic disease: granulomatosis with polyangiitis (n = 3), Behçet's disease (n = 2), unspecified inflammatory arthritis (n = 2), psoriatic arthritis (n = 1), ankylosing spondylitis (n = 1), sarcoidosis (n = 1), Cogan's syndrome (n = 1) and ulcerative colitis (n = 1). Six patients (18.8%) were suspected of having infectious disease with herpes virus: clinical context and positive treatment response with oral valacyclovir. Systemic agents and topical agents were required in 28 patients (87.5%). The first line therapy was mainly oral non-steroidal anti-inflammatory drugs in 15 patients (47%) and oral corticosteroids in 8 (25%). Immunosuppressive drugs were required in 6 patients. The mean follow-up was 16.3 months. Six patients (19%) had a decrease in visual acuity.

Conclusion

The number of systemic disease in our series is similar to the main series in the literature. Treatment with valaciclovir might be effective in patients with suspected herpes simplex scleritis.     

Soins palliatifs chez les patients en insuffisance cardiaque terminale

Purpose

Heart failure is a common disease and its progression to end-stage heart failure is responsible of high mortality. The aim of this retrospective study was to assess the access to integrated palliative care to the usual management, 6 months prior to their death, and especially during the last hospitalization.

Patients and methods

A retrospective study was performed in patients who died of heart failure in 2009 in two hospitals. The analysis was performed on 20 cases of each institution. The records of consecutive patients were included in an anti-chronological order from 31st December 2009.

Results

For their last hospitalization, 37 patients (93%) were hospitalized in emergency. Within 3 days prior to death, the most frequent symptoms were dyspnea (n = 33, 82%), and pain (n = 30, 75%). Therapeutic most frequently used were oxygen (n = 31, 77%) and analgesics (n = 30, 75%). No patient was seen by a psychologist. The decision to limit treatment for comfort care was reported for 24 patients (60%) and the median of the average time between the decision and death was 2 days (Q1–Q3, 1–5 days).

Conclusion

Patients with terminal heart failure have many symptoms often requiring multidisciplinary care. This type of study relating practices shows that there is still a lot to do to integrate palliative care in the usual management of patients with heart failure.     

La pancréatite lupique : une série de six cas

Purpose

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.

Methods

This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3), and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.

Conclusion

Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.     

Transient variations of transthoracic impedance as a predictor of heart failure and death in patients with implanted defibrillators

Background

Transient variations in physiological parameters may forewarn of life-threatening cardiac events, but are difficult to identify clinically. Implantable cardioverter defibrillators (ICD) designed to measure transthoracic impedance provide a surrogate marker for pulmonary congestion.

Objective

The aim of this study is to determine if the frequency of changes in transthoracic impedance (TTI) is associated with congestive heart failure (CHF) exacerbation and predicts mortality.

Methods

We followed 109 consecutive patients (pts) with ICDs (n = 58) or CRT-ICDs (n = 51) for a mean of 21.3 (+ 10.2) months. Using 80 ohm-days as a reference, we correlated the frequency of TTI changes above this index to CHF hospitalizations or death.

Results

There was at least one TTI threshold crossing in 79 (72%) pts over 23.3 months follow-up, with a mean of 1.8 ± 3.4 per year. There were 18 pts with CHF hospitalizations who had a mean of 4.3 TTI threshold crossings/year (S.D. = ± 7.3; median = 2.8), compared to 1.3 (S.D. = ± 1.5; median = 0.8) among pts without CHF hospitalizations (p = 0.0006). Among 20 patients who died during follow-up, there were 4.2 (S.D. = ± 7.0; median = 2.9) TTI threshold crossings/year, compared with 1.3 (S.D. = ± 1.3; median = 0.9) threshold crossings/year among survivors (p = 0.0004). Using Cox Proportional Hazard modeling, after adjusting for age, baseline EF, and number of shocks, TTI threshold crossing was an independent predictor of death (HR 1.72, 95% CI 1.26–2.36, p = 0.001).

Conclusions

Increased frequency of TTI threshold crossings may be a useful predictor of transient risk for identifying a subgroup of ICD recipients at greater individual risk for death or CHF hospitalizations.     

Myopathies inflammatoires et anticorps anti-PM-Scl : à propos d’une série et revue de la littérature

Purpose

The objectives of this study were to evaluate: (1) the prevalence of anti-PM-Scl antibodies within the framework of antinuclear antibodies detection; and (2) the clinical features and outcome of patients with isolated polymyositis/dermatomyositis.

Methods

Nine thousand and sixty-four consecutive antinuclear testing data allowed us to evaluate anti-PM-Scl antibody prevalence. Second, we also assessed the characteristics of patients with isolated dermatomyositis/polymyositis and associated anti-PM-Scl antibody.

Results

Over 9064 consecutive antinuclear samples tested for antinuclear antibodies, 3263 (36%) were positive; anti-PM-Scl antibody were positive in nine patients: 0.1% of all sera, 0.2% of sera positive for antinuclear antibodies, 1.2% of sera positive for anti-ENA antibodies. Four of the nine patients with anti-PM-Scl antibody had dermatomyositis (n = 3) and polymyositis (n = 1). Patients with dermatomyositis/polymyositis and anti-PM-Scl antibody exhibited severe complications, as follows: ventilatory insufficiency (n = 2) requiring mechanical ventilation in one case, esophageal involvement requiring enteral feeding (n = 1); also, two of these patients had cancer.

Conclusion

Our case series suggests that the presence of anti-PM-Scl antibody is not a favorable prognostic factor in patients with dermatomyositis/polymyositis. This type of antibody appears to be associated with lung and esophageal involvement; in addition, anti-PM-Scl antibody may co-exist with malignancy in PM/DM patients. Taken together, we suggest that patients with dermatomyositis/polymyositis and anti-PM-Scl antibody require both initial evaluation for lung/digestive manifestations and cancer and close surveillance.     

Prévalence et valeur diagnostique des anticorps antinucléaires de spécificité antigénique indéterminée : étude rétrospective à propos d’une série de 90 patients

Purpose

The objective of this study was to determine the clinical relevance and the diagnostic significance of positive antinuclear antibodies (ANA) without identified antigenic target by the usual characterization technique.

Patients and methods

Retrospective study conducted in the Laboratory of Immunology of Habib Bourguiba Hospital (Sfax, Tunisia) during 18 months. The inclusion criteria were the presence of an ANA titer greater or equal to 1/320 with negative characterization result. ANA screening was performed by indirect immunofluorescence (IIF) on Hep2 cells. Each positive serum was tested by IIF on Crithidia luciliae (anti-native DNA) and by immunodot (anti-nucleosome, anti-histone, anti-Sm, anti-RNP, anti-SSA, anti-SSB, anti-Scl 70, anti-PM-Scl, anti-Jo1, anti-PCNA and anti-ribosomal protein). Sera of systemic lupus erythematosus (SLE), myositis, and scleroderma patients were tested for anti-Ku, anti-PL7, anti-PL12 and anti-Ro-52 using dot myositis.

Results

Sera of 90 patients were studied: 18 men and 72 women (average age: 44 years). Drug-induced ANA was found in eight patients. The most frequent clinical symptoms were joint (56.7%), cutaneous (54.4%) and constitutional symptoms (45.6%). The diagnosis of an autoimmune disease was suspected in 49 patients (54.5%) and confirmed in 30 (33.3%) including 20 cases of connective tissue disease: myositis (n = 6), scleroderma (n = 5), Sjögren's syndrome (n = 3), SLE (n = 4), rheumatoid arthritis (n = 6) and antiphospholipid syndrome (n = 4). Other autoimmune diseases were less frequent. The anti-Ku antibody was detected in the majority of patients with connective tissue disease. The diagnosis of non-autoimmune diseases was established in 25.5% of patients. Eighteen patients (20%) had no diagnosis orientation.

Conclusion

Our study demonstrated the diagnostic value of the presence of ANA even in the absence of known antigenic target, confirmed the role of the IIF as “gold standard” test for ANA screening, and suggested the usefulness of the addition of Ku antigen in the immunodot classic profile.     

Beneficio clínico de la terapia de resincronización cardiaca con desfibrilador para pacientes con fracción de eyección > 35% por resonancia magnética

Introduction and objectives

Cardiac resynchronization therapy with a defibrillator prolongs survival and improves quality of life in advanced heart failure. Traditionally, patients with ejection fraction > 35% estimated by echocardiography have been excluded. We assessed the prognostic impact of this therapy in a group of patients with severely depressed systolic function as assessed by echocardiography but with an ejection fraction > 35% as assessed by cardiac magnetic resonance.

Methods

We analyzed consecutive patients admitted for decompensated heart failure between 2004 and 2011. The patients were in functional class II-IV, with a QRS ≥ 120 ms, ejection fraction ≤ 35% estimated by echocardiography, and a cardiac magnetic resonance study. We included all patients (n = 103) who underwent device implantation for primary prevention. Ventricular arrhythmia, all-cause mortality and readmission for heart failure were considered major cardiac events. The patients were divided into 2 groups according to systolic function assessed by magnetic resonance.

Results

The 2 groups showed similar improvements in functional class and ejection fraction at 6 months. We found a nonsignificant trend toward a higher risk of all-cause mortality in patients with systolic function ≤ 35% at long-term follow-up. The presence of a pattern of necrosis identified patients with a worse prognosis for ventricular arrhythmias and mortality in both groups.

Conclusions

We conclude that cardiac resynchronization therapy with a defibrillator leads to a similar clinical benefit in patients with an ejection fraction ≤ 35% or > 35% estimated by cardiac magnetic resonance. Analysis of the pattern of late gadolinium enhancement provides additional information on arrhythmic risk and long-term prognosis.Full English text available from:www.revespcardiol.org/en     

Uvéites révélant une sarcoïdose : caractéristiques cliniques à propos de 23 cas

Purpose

Uveitis may rarely reveal sarcoidosis in Caucasian patients. Our objective was to analyze the clinical manifestations, and the outcome in a group of patients in whom uveitis was the presenting manifestation of sarcoidosis.

Methods

Retrospective study including 23 patients (mean age: 50.3 ± 14.5 years) diagnosed with sarcoidosis after an episode of uveitis. Granulomatous lesions were documented in 14 patients.

Results

Ophthalmological examination revealed anterior uveitis (n = 5), intermediate uveitis (n = 2), posterior uveitis (n = 25) and panuveitis (n = 11). Ocular inflammation was bilateral in 16 patients (69,6%), typical aspects of granulomatous uveitis were found in only 16 eyes over 39 (41%), posterior uveitis was found in 18 eyes (46.2%), with an averaged visual acuity of 5/10. Macular oedema was noted in five patients. Suggestive signs of ocular sarcoidosis were present in 43% of the patients. Stage 1 or 2 pulmonary involvement (n = 22), musculoskeletal (22%), skin (13%), or spleen (9%) involvements were the most common findings. Oral corticosteroids were necessary in 91.3% of the patients, immunosuppressive agents in 26.1%, with a prolonged treatment greater than two years in 58%. The visual prognosis was good, with visual acuity greater than 6/10 in 96% of the cases if the ocular inflammation spared retina and choroid. However, a visual acuity less than 6/10 was observed in 44% of the cases when the posterior segment was involved.

Conclusion

Sarcoidosis may be revealed by an intraocular inflammation, with typical patterns in only 43% of the cases. Sarcoidosis should therefore be included in the differential diagnosis of every uveitis. Oral corticosteroids are required in almost all cases, owing to ocular involvement rather than visceral involvement.