Oral involvement in sarcoidosis: report of 12 cases.

AIM: To assess the clinical features, treatment and outcome of oral sarcoidosis and to determine whether oral involvement is associated with a particular clinical phenotype of sarcoidosis. DESIGN: Multicentric retrospective study. METHODS: Retrospective chart review. Each patient was matched with four controls. RESULTS: Twelve patients (9 women, 3 men) were identified. Their median age at sarcoidosis diagnosis was 38 years. Oral involvement was the first clinical evidence of sarcoidosis in seven cases and was a relapse symptom in five cases. Clinical presentations were nodules (n = 7) or ulcers (n = 5) and were mostly solitary. The tongue was the commonest site affected (n = 4), followed by lips (n = 3), oral mucosa (n = 2), palate (n = 2) and gingiva (n = 1). Patients with oral sarcoidosis were significantly younger and had more frequent lacrimal or salivary glands and upper airway tract clinical involvement than the controls; increased angiotensin-converting enzyme was less frequent in oral sarcoidosis. Multiple treatments of oral sarcoidosis were used: no treatment (n = 3), surgery (n = 2), corticosteroids (n = 7), hydroxychloroquine (n = 3), methotrexate (n = 2), doxycycline (n = 1). Methotrexate was efficient in one patient, hydroxychloroquine showed benefit in only 1 out of 3 patients. Three patients presented oral relapses. After a mean follow-up of 6 years, 10 patients experienced a complete (n = 7) or partial (n = 3) remission of oral sarcoidosis; stability was observed in the remaining two cases. CONCLUSION: Although oral manifestations of sarcoidosis are unusual, physicians should be aware that this specific localization is frequently the first manifestation of the disease. Treatment modalities range from observation in asymptomatic patients to immunosuppressants for severe involvement.

[Severe extrathoracic sarcoidosis]. / Localisations extrathoraciques graves de la sarcoïdose.

Sarcoidosis may affect functional outcome and sometimes may be a life-threatening condition. Functional outcome is particularly compromised by cutaneous, ocular, nasal, osteoarticular manifestations and some neurological expression. Central nervous system involvement and cardiac sarcoidosis could be life-threatening, as laryngeal and renal involvement. Diagnosis is sometimes very difficult to obtain. Several tissue biopsies are usually necessary to rule out all differential diagnosis. Extrathoracic manifestations of severe sarcoidosis require a corticosteroid therapy, initiated, if life-threatening at a daily dose of 1 mg/kg, usually associated with an other immunosuppressive drug (methotrexate, cyclophosphamide, anti TNFα).

[Neurosarcoidosis]. / Neurosarcoïdose: état des connaissances.

The origins of neurosarcoidosis, a multisystemic granulomatous disease, remain unknown. Nervous system localizations remain rare, but severe. Lymphocytic meningitis, psychiatric disorders, diabetes insipidus and cranial nerve palsy are the most frequent signs. Cerebral fluid test and cervical medullar and cerebral MRI with gadolinium have to be performed first. In some cases, histological evidence of granuloma have to be obtained with neuromuscular, meningeal or cerebral biopsies. Functional impairment and life-threatening conditions require early corticosteroid therapy. In worsening cases or in the event of no therapeutic response or poor tolerance to corticosteroids, other immunosuppressive agents should be associated. Maintenance therapy and most often life long maintenance therapy allow a continuous success while avoiding relapse.

[Bilateral optic neuropathy revealing systemic sarcoidosis]. / Neuropathie optique bilatérale révélatrice d'une sarcoïdose systémique.

INTRODUCTION: Although ocular involvement concerns about 25 to 60% of patients with systemic sarcoidosis, papilledema is a rare event. EXEGESIS: A 43-year-old man presented with left visual loss related to papillitis. Despite high-dose corticosteroids, visual loss became rapidly bilateral. Thoracic CT-scan showed mediastinal and parenchymal involvement. Histological study of lung, bronchial and lymphadenopathy biopsies displayed giant-cells granuloma without necrosis. Diagnosis of sarcoidosis complicated with optic neuropathy was made. Therapy combining prednisone, methotrexate and infliximab was started without significant improvement of visual acuity. CONCLUSION: This report underlines the potential severity of optic neuropathy in sarcoidosis.

[Epidemiology of sarcoidosis and its genetic and environmental risk factors]. / Epidémiologie de la sarcoïdose et ses facteurs de risque génétiques et environnementaux.

PURPOSE: Review of the literature on epidemiologic data of sarcoidosis and risk factors. CURRENT KNOWLEDGE AND KEY POINTS: Epidemiological data show that sarcoidosis is a world-wide disease. Frequency is influenced by multiple predisposition factors. The most important are racial factor, sex, age, familial aggregation, genetic factor and/or infective agent. One of these is insufficiency. FUTURE PROSPECTS AND PROJECTS: Patent sarcoidosis is revealed when concomitant environmental factors (infective agent, climate, country) and predisposition ones (race, sex familial aggregation) appear together and inducing, in each patient, a particular sarcoidosis.

[Neurosarcoidosis]. / Neurosarcoïdoses.

A neurological localization is observed in 20% of the cases of sarcoidosis. Involvement of the central and/or peripheral nervous system is generally observed in Caucasians while cranial nerve localization predominates in blacks. Beside these particular elements, lymphocytic meningitis, psychiatric disorders, insipid diabetes, and cranial nerve palsy are the most frequent signs. A cerebrospinal fluid test as well as brain and spinal cord MRI with gadolinium injection is required in all cases. Depending on the clinical expression, complementary tests may include PEA, PEV and neuropsychic tests. Histological proof of sarcoidosis granuloma is required for diagnosis but may be difficult to obtain when neurological signs are not associated with another localization. Systemic treatment is indicated, based on steroids, sometimes associated with another immunosuppressive agent. After acute treatment, chronic therapy must be maintained for years, and sometimes for life.

[Cardiac sarcoidosis]. / Sarcoïdoses cardiaques.

A cardiac localization is one of the most severe manifestations of sarcoidosis and may cause sudden death (ventricular tachycardia or atrial ventricular block III) or restrictive cardiomyopathy. Lesions are most frequently observed in the interventricular septum and the free left wall. Granulomatous infiltation can provoke nonspecific clinical, electric and echocardiographic signs, which, associated with regressive dipyridamol uptake on tomoscintigraphy, are suggestive of cardiac sarcoidosis. The diagnosis of cardiac sarcoidosis is based on the presence of systemic sarcoidosis, histological evidence of granuloma and the lack of another cause of cardiomyopathy. Corticosteroid therapy is indicated, associated with specific cardiologic treatments.

[Neurologic forms of sarcoidosis]. / Les formes neurologiques de la sarcoïdose.

BACKGROUND: Neurosarcoidosis is an uncommon but severe, sometimes life threatening, manifestation of sarcoidosis. Signs of neurological involvement usually are seen in patients known to have active disease. Strictly neurological forms are seen in less than 10% of cases. CLINIC: Neuropsychic manifestations are the most common clinical signs, independent of corticosteroid therapy or neuroendocrine involvement, epileptic seizures, and signs related to hypocephalia. DIAGNOSIS: Diagnosis of systemic sarcoidosis is confirmed on the basis of clinical arguments and laboratory findings favoring neurosarcoidosis. Key investigations include angiotensin converting enzyme assay in cerebrospinal fluid, and brain stem magnetic resonance imaging with gadolinium injection. Nerve biopsies may be needed in certain cases. TREATMENT: Corticosteroid therapy is given as first line treatment with a satisfactory effect in most cases. Immunosuppressors may be added in case of failure.

Central nervous system sarcoidosis: follow-up at MR imaging during steroid therapy.

PURPOSE: To document the changes observed at sequential magnetic resonance (MR) imaging of sarcoidosis lesions of the central nervous system (CNS) during treatment with corticosteroids. MATERIALS AND METHODS: The abnormalities detected in 24 patients (mean follow-up, 36 months) were compared before and after therapeutic periods (n = 75) that were divided into attack (high-dose), upkeep (decreased-dose), and minimal (low-dose) periods. Parenchymal lesions were classified as type 1 (enhanced with gadolinium), type 2 (demyelinating), or type 3 (lacunar) and were assessed as regressing, stable, or progressing. RESULTS: Seven of the 24 patients had several types of lesions. Isolated type 3 lesions (six patients) were the only lesions not associated with neurologic deficit. Type 1 lesions (13 patients) regressed in 22 of 22 attack periods and progressed in nine of 27 upkeep and minimal periods. MR imaging depicted relapses in patients with multifocal CNS involvement or long-standing CNS impairment or in those who had previously received steroid therapy. Type 2 (seven patients) and type 3 (13 patients) lesions remained stable in 68 of 68 therapeutic periods. Type 1 lesions appeared in three patients with type 2 and type 3 lesions during two upkeep and three minimal periods. Findings at follow-up MR imaging contributed to the reintroduction of high-dose corticosteroid therapy in eight patients. CONCLUSION: MR imaging can be used to differentiate between reversible and irreversible lesions in CNS sarcoidosis. MR imaging can be a useful tool for adjusting treatment to prevent irreversible CNS damage.

[Sarcoidosis and pregnancy. A retrospective study of 11 cases]. / Sarcoïdose et grossess. Etude retrospective de 11 cas.

PURPOSE: To analyze the evolutive profile of sarcoidosis together with reciprocal interactions between pregnancy and sarcoidosis. METHODS: All events that occurred during pregnancy in 11 women presenting with sarcoidosis were analyzed. Histological confirmation was obtained for the 11 cases. For all pregnancies were analyzed the course of both sarcoidosis and pregnancy, and the influence of pregnancy on the disease evolution. RESULTS: Among 33 pregnancies, 23 led to the birth of healthy fetuses (five spontaneous abortions, four voluntary abortions, and one therapeutic abortion). The major event was fetal hypotrophy in six cases. Three of them occurred during pregnancy in prednisone-treated patients with active sarcoidosis. No relapse of cured sarcoidosis or further evolution of sarcoidosis that was inactivated as of the beginning of pregnancy were observed. The course of active sarcoidosis varied, as improvement (one case), worsening (two cases) and stabilization (two cases) were observed. During the first year of follow-up after delivery, four relapses and, in two cases, preliminary signs of the disease were observed. CONCLUSION: Apart from the hypothetical but not definite risk of hypotrophy, no negative interaction between sarcoidosis and pregnancy could be established. Pregnancy does not seem to interfere with the course of sarcoidosis. Considering the risk of relapse after delivery, pregnant women presenting with sarcoidosis should benefit from clinical and radiological follow-up.

[Sarcoidosis of the central nervous system]. / Sarcoïdose du système nerveux central.

PURPOSE: Current knowledge on central nervous system sarcoidosis. CURRENT KNOWLEDGE AND KEY POINTS: Sarcoidosis is localized in the central nervous system in 5 to 16% of the cases. Various neurological manifestations are observed, including: seizures, cognitive or psychic manifestations, hypothalamic and pituitary involvement, local pseudotumors, and hydrocephalus very frequently associated with asymptomatic lymphocytic meningitis and with cranial nerve palsy, particularly palsy of the seventh nerve, occurring less regularly. CNS localization is most often an early manifestation of the disease, unmasking sarcoidosis. It is often part of primary or secondary systemic polyvisceral sarcoidosis. The diagnosis is mainly based on two arguments: confirmation of the existence of systemic sarcoidosis and clinical and paraclinical compatibility of neurological abnormalities (particularly at magnetic resonance imaging [MRI]). Neurological histopathology is rarely necessary to confirm the diagnosis. Corticotherapy is indicated in all symptomatic cases and most often leads to a more or less complete clinical response evidenced by regression of active lesions identified on MRI. The treatment must often be prolonged for several years, and clinical and MRI evolution help guide therapeutical choices for dosages and threshold doses. CNS involvement is potentially severe with mortality and morbidity rates that are not insignificant. Limiting of iatrogenic risks requires adequate follow-up. PERSPECTIVES AND PROJECTS: Multicenter studies are necessary to determine factors influencing the incidence and long-term prognosis of CNS sarcoidosis treated with corticotherapy. The efficacy of treatments other than corticotherapy must be evaluated.

[Magnetic resonance angiography: fields of exploration, main indications and limitations]. / L'angiographie par résonance magnétique: domaines d'exploration, principales indications et limites.

Magnetic resonance angiography (MRA) has become a widely accepted technique with regards to the other available noninvasive techniques in the diagnosis of vascular disease. This paper proposes a review of the different indications of this technique in arterial and venous diseases. Among several MRA pulse sequences, the most frequently used until today consisted of a time-of-flight technique which provided angiograms without any injection of intravascular contrast medium. It required to be performed in a plane perpendicular to the main axis of the vessel to be optimal. New techniques, such as contrast medium bolus-enhanced acquisitions allow examination of vascular segments in a plane parallel to their course (coronal for the aorta and lower limb arteries). An increasing number of clinical applications has raised since the implementation of MRA techniques on MR devices; some of them are widely accepted, whereas some others remain under the scope of extensive validation. With a high level of accuracy in grading carotid artery stenosis, MRA is now routinely used in cerebral arterial occlusive diseases and has in part replaced contrast angiography. MRA of the venous system of the brain plays a major role in the diagnosis and follow up of dural venous thrombosis. Other vascular brain diseases, such as vascular malformations, yet have limited uses. Carotid artery dissections are fairly demonstrated with MRA, which can be used for diagnosis as well as for follow-up. The accuracy of MRA in the diagnosis of venous thrombosis of the cervical/mediastinal veins has been reported as high as 100%. Moreover, MRA allows a precise assessment of collateral vessels in case of complete cervical/mediastinal venous thrombosis.

[Sarcoidosis symptomatic cardiomyopathy: report of 10 cases]. / Cardiomyopathies sarcoïdosiques symptomatiques: à propos de 10 cas.

This study illustrated 10 cases of sarcoidosis symptomatic cardiomyopathy. All sarcoidosis were confirmed by histology. One was revealed by cardiological signs. Extra-cardiologic manifestations were non specific, as well cardiological and electrical signs. ECG were abnormal in only 70% of cases. Echocardiography and thallium scintigraphy with dipyramidole test were always abnormal. No sudden death appeared in our series. As opposed to all cases in literature, all patients, but one, responded positively to corticotherapy. These results can be explained by high doses of prednisolone associated, when non effective, to another immunosuppressive agent.

[Rare sites of sarcoidosis: clinical aspects and reflections on the diagnostic approach]. / Les localisations rares de la sarcoïdose: aspects cliniques et réflexions sur l'approche diagnostique.

Rare localisations of sarcoidosis, mainly neurological and cardiological ones are in the most cases seriously threatening whether functional or vital prognosis. They are often responsible for a delay in diagnosis or treatment. Concerning young patients, any unexplained neurological or cardiological manifestations should lead to consider the diagnosis of sarcoidosis. When histological evidence is not obtained, in particular in presence of central nervous system localisations. Systemic explorations such as pulmonary radiography, bronchoalveolar lavage, salivary glands biopsies should be performed. In most cases, these localisations will lead to corticotherapy, which, when revealing unsuccessfully, is associated with another immunosuppressive agent.

[Severe extrapulmonary forms of sarcoidosis]. / Formes extrapulmonaires graves de la sarcoïdose.

Severe extrapulmonary sarcoidosis mainly consists of cardiac and neuromuscular involvement, both of which compromise the functional prognosis and survival. Their rare occurrence is a major factor in delayed diagnosis and treatment when they are the presenting signs of the disease, and especially when not associated to the "classic" signs of sarcoidosis. The lack of clinical and paraclinical specificity hinders diagnosis, and in the absence of histological evidence, all clinical and paraclinical factors compatible with sarcoidosis must be taken into account. Since these localisations are often asymptomatic or poorly symptomatic, they should be routinely sought by careful clinical examination and electrocardiogram. The presence of such signs modifies the therapeutic approach and requires treatment with corticosteroids.