Sclérites et épisclérites : prise en charge diagnostique et thérapeutique

Episcleritis and scleritis are distinct entities with regard to visual prognosis, risk of associated systemic disease, and treatment. The pertinence of the clinical classification of episcleritis and scleritis established in 1976 still persists, with significant differences in terms of visual prognosis, associated general conditions, and therapeutic choices according to each scleritis subtype. Episcleritis requires rarely to be referred to a tertiary care centre, and if so it has to be monitored similarly to scleritis. In this paper, an analysis of 1358 scleritis cases from the main distinct large series published since 1976 shows a mean proportion of 8% of infectious aetiologies (mainly herpes viruses), and 28% of systemic diseases with two main subgroups: inflammatory rheumatisms 12.8%, and systemic vasculitis 7.8%. Overall, the risk for visual loss following scleritis is around 16%. However, the risks of associated systemic disease and visual loss are both highly variable according to the type of scleritis, and culminate at 80% and 50% in the necrotizing subtype respectively. As compared with older series, the proportion of necrotizing scleritis is lower in recent series which is likely due to the advances obtained over the past 20 years in immunomodulatory therapy, as well as its wide use in the treatment of the main systemic conditions associated with scleritis. The treatment of scleritis should be managed by physicians who are experts in the use of immunosuppressive drugs that may be required in one out of two affected patients.     

Amylose cardiaque : à propos d’une série de 14 patients,description et facteurs pronostiques

Purpose

Cardiac amyloidosis is rare. The objective of this study was to report on a case series of 14 patients with cardiac amyloidosis and to study the prognostic factors.

Methods

Monocentric retrospective study of all adult patients who presented with cardiac amyloidosis, diagnosed at the Georges-Pompidou European hospital in Paris between 2003 and 2011.

Results

Fourteen patients were identified (10 men and four women). Median age at diagnosis was 66.5 years. Twelve patients were diagnosed with AL amyloidosis, one with AA amyloidosis, and one with transthyretin amyloidosis. All patients presented cardiac manifestations: heart failure (n = 9), rhythm disorders (n = 6). Eight patients presented extra-cardiac manifestations of amyloidosis: renal (n = 8), gastrointestinal (n = 5). Troponin serum level was increased in eight patients and BNP level was superior to 400 pg/L in 12 patients. When performed, the cardiac magnetic resonance imaging (MRI) showed, in six patients out of seven, chamber dilatation, concentric hypertrophy or late enhancement. Among patients with cardiac failure at diagnosis (n = 9), seven died with a median survival of 1 month duration. Factors of poor prognosis were, in our study, heart failure, elevated levels of troponin and BNP, and the AL amyloidosis subtype.

Conclusion

Cardiac amyloidosis, especially the AL type, has a very poor prognosis, essentially because of an underlying multiple myeloma and heart failure.     

Aspects cliniques et étiologiques des uvéites : étude rétrospective de 121 patients adressés à un centre tertiaire d’ophtalmologie

Purpose

Uveitis consists of a large group of diseases characterized by intraocular inflammation involving the uveal tract. This heterogeneity makes the diagnosis and the treatment of uveitis frequently challenging. The purpose of this study was to describe the various clinical and etiologic aspects of uveitis, through the new standardized uveitis classification and the use of modern investigations for its diagnostic work-up.

Methods

The medical records of 121 new patients with uveitis referred to our tertiary ophthalmologic centre between January 2002 and December 2006 were retrospectively reviewed. Uveitis associated to human immunodeficience virus and secondary to exogenous endophthalmitis were excluded. All patients had a complete ophthalmological examination and appropriate clinical and paraclinical examination. The diagnosis was established according to the recent international criteria.

Results

One hundred and twenty-one patients were included. The four main etiologies were: toxoplasmosis (14%), sarcoidosis (11.6%), spondylarthritis or HLA B27-associated uveitis (13.2%) and Herpes virus infections (9.1%) that represented almost half of the uveitis causes (47.9%). Various diseases constituted the remaining causes of the uveitis (20.9%). Uveitis remained unexplained in the remaining 36 patients (29.7%). Overall, associated systemic diseases were diagnosed in 35.5% of our uveitis patients (34 patients), associated infectious conditions in 26.4% (32 patients) and specific ocular diseases in 8.3% (12 patients).

Conclusion

Despite a limited number of patients, our study showed an etiologic distribution similar to that of the main series reported in the literature. Nevertheless, we observed an elevated frequency of sarcoidosis and systemic diseases, which emphasizes a management that takes into account standardized clinical and paraclinical criteria and the usefulness of a collaboration with the internist.     

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.     

Atteinte du sinus caverneux au cours d’une granulomatose avec polyangéite (maladie de Wegener) sans ANCA

Introduction

Granulomatosis with polyangeitis (Wegener's granulomatosis) (GPA) is a granulomatous vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA). Affected organs usually include upper and lower respiratory tracts, and kidneys. Limited forms of GPA may affect the central nervous system (vasculitis, hypertrophic pachymeningitis, encephalitis), a location in which diagnosis is often uneasy.

Case report

We report a 74-year-old woman who presented with a limited form of GPA affecting the cavernous sinus. Diagnosis was considered in view of a retrospectively suggestive clinical presentation, compatible cerebral MRI and temporal artery biopsy, despite the absence of ANCA. It was supported by a favourable outcome with cyclophosphamide administration.

Conclusion

GPA presenting as a cavernous sinus syndrome is rare. Three co-existing pathogenic mechanisms may be involved in GPA affecting the central nervous system: contiguous invasion from nasal or orbitary granulomatous sites, vasculitis, or primary intra-cerebral granulomatous inflammation. Lack of biopsy evidence of affected tissues and ANCA negativity should not delay diagnosis and appropriate therapeutic management in central nervous system GPA.     

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.     

Infarctus du myocarde au cours d’une activité sportive. Caractéristiques cliniques et angiographiques en phase aiguë d’une série de 16 cas

Objective

Determination of clinical and angiographic characteristics of myocardial infarctions related to sport.

Methods

Retrospective study of acute coronary syndromes with ST elevation related to sport treated with interventional cardiology from 2006 to 2013.

Results

Sixteen patients were included. They are mostly men (15/16), aged 24–65 years (over 35 years old in 13 cases) with few cardiovascular risk factors, most frequently heredity or smoking. Myocardial infarctions usually occur during the practice of sports (13/16), with serious rhythmic complications in three of the cases. On angiography, most patients have single vessel disease (12/16).

Conclusion

Myocardial infarction related to sports affects a male population aged over 35 years old with few cardiovascular risk factors, most often single vessel disease, making the preventative screening uneasy. Other studies investigating larger populations, assessing previous clinical events (symptoms, results of stress tests), evaluating the impact of competition and integrating sudden deaths would improve the screening and the treatment of sport-related myocardial infarctions.     

Apport de l’IRM cardiaque dans l’évaluation initiale et le suivi des myocardites mimant un syndrome coronaire aigu : à propos d’une série de 43 patients

Aims

The diagnosis of acute myocarditis is complex, especially when the clinical presentation mimics an acute coronary syndrome. This condition may promote the progression to dilated cardiomyopathy and the occurrence of severe arrhythmias. A reassessment integrating a cardiac MRI at three months after the acute episode could help identify patients with a poor prognosis.

Patients and results

This prospective series of 43 consecutive patients hospitalised for acute myocarditis included 36 men and seven women, with a mean age of 32 years, with no indication of heart failure. All patients presented elevated levels of troponin I. Echocardiography showed moderate left ventricular dysfunction in six cases and segmental wall motion abnormalities in 22 cases. After gadolinium injection, a subepicardial late enhancement was observed in 39 cases. Three months after the acute episode, all patients were asymptomatic. The echocardiography and laboratory tests were normal. In 23 cases, the MRI showed persistence of the late enhancement without segmental wall motion abnormality. After a mean follow-up of three years, one patient was lost to follow-up and only one suffered a heart failure revealing a dilated cardiomyopathy complicated by ventricular arrhythmias.

Conclusion

On admission, the subepicardial localisation of late enhancement in the cardiac MRI is reliable criteria for the diagnosis of acute myocarditis, enabling to rule out an acute coronary syndrome. During follow-up, the persistence of late enhancement has no impact on prognosis. In this series, after a mean follow-up of three years, it was not associated with clinical or paraclinical abnormalities, except in one patient.     

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.     

Caractéristiques cliniques,paracliniques et profil évolutif de l’atteinte aortique de la maladie de Horton : à propos de 26 cas d’aortite parmi 63 cas de maladie de Horton

Purpose

Aortic involvement that occurs in temporal arteritis is probably underestimated because it is usually asymptomatic. The characteristics of giant cell arteritis with aortic involvement are still poorly described and the relationship between aortitis and vascular outcome of the disease has not been clearly delineated. The objective of this retrospective study of 63 patients with giant cell arteritis, including 26 with aortic involvement, was to compare the features of patients with and without aortitis, and to assess the contribution of CT-scan and FDG-PET-scan in screening for vascular disease, monitoring, and therapeutic management of patients.

Methods

This retrospective study was conducted in the internal medicine department of the university hospital in Marseille, France, from January 1, 2005 to September 30, 2011. Patients had at least three out of the five American College of Rheumatology criteria for temporal arteritis and aortic involvement was investigated in all patients using CT-scan. Aortic wall thickness greater or equal to 3 mm was considered to be abnormal.

Results

Of 63 patients diagnosed with giant cell arteritis, 26 (41.3%) had aortic involvement diagnosed by aortic CT-scan. Age at diagnosis was significantly younger (66.8 vs 73.8 years; P = 0.002) in the group with aortitis. Inflammatory dorsal and low back pain, signs of vascular disease of the upper limbs (P = 0.009), and higher level of acute phase reactants were associated with aortitis. Aneurysmal lesions of the aorta were significantly more frequent in the group with aortitis. Twenty patients had both aortic CT-scan and FDG-PET-scan. For patients in whom aortic involvement was not demonstrated with CT-scan, FDG-PET-scan was always non-contributive. With corticosteroids, aortitis resolved within 6 months in all patients as evaluated by aortic CT-scan. However, aortitis persisted in 80% of cases at 6 months when evaluated with FDG-PET-scan, and in 66% of cases at 12 months, without influencing the treatment.

Conclusion

This case series shows no specific features of aorta and its main roots involvement in giant cell arteritis, justifying a systematic screening by CT-scan. The high frequency of this arterial involvement could help physicians in the diagnosis of giant cell arteritis. Aortitis seems to be associated with vascular complications as highlighted by the frequency of aortic aneurysm and a case of early aortic dissection. Finally, the role of PET-CT-scan for screening vascular disease and therapeutic monitoring remains to be clarified.     

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.     

L’artérite de Takayasu : mise au point à propos d’une série monocentrique de 82 patients

Takayasu arteritis (TA) is an uncommon large-vessel arteritis. We report our French single-center experience in the management of patients with TA (Pitié-Salpêtrière Hospital, Paris). TA is diagnosed in patients presenting with a large-vessel arteriopathy, in whom several inflammatory and non-inflammatory differential diagnoses are ruled out by appropriate investigations. Treatment of active disease is primarily based on corticosteroids but other immunosuppressive drugs are frequently needed. Anti-platelets agents, statins and antihypertensive drugs are frequently considered. There is no validated disease activity criterion in TA; thus, we generally consider the disease as being active in the presence of the following criteria: firstly, the presence of constitutional or ischemic symptoms; secondly, the increased acute phase reactants; thirdly, the mural contrast enhancement in CT-scan or fourthly, the mural contrast enhancement or signal abnormalities in MRI; fifthly, the abnormal vascular uptake in PET-scan. When TA is active, our follow-up recommendation is to perform an ultrasonography of the supra-aortic vessels and an aortic MRI or CT-scan twice a year. When TA appears to be inactive, we recommend to perform these investigations once a year. Surgical treatment of TA is limited to a few indications. The overall prognosis of TA is good but the quality of life is altered. Management of TA patients is difficult because of the lack of reliable diagnostic criteria, consensual therapeutic strategies and validated disease activity criteria. Further studies should focus on the pathogenesis of the disease and help define better disease activity criteria.     

Paralysie du nerf récurrent au cours d’une maladie de Lyme : à propos de deux observations

Introduction

Neuroborreliosis can be a difficult diagnosis which requires epidemiologic, clinical and biologic arguments.

Case reports

We report two patients who presented with a recurrent laryngeal nerve palsy with positive Lyme serology and favorable outcome after antibiotic therapy. In one case, a lymphocytic meningitis with intrathecal production of specific antibodies was evidenced.

Conclusion

Recurrent laryngeal nerve palsy is an uncommon manifestation of neuroborreliosis. Lyme serology is an important tool when neurologic disorder occurs because of an atypical course of Lyme disease.