Syndrome de Sweet révélant une polychondrite atrophiante

Relapsing polychondritis (RP) is a rare disorder characterized by recurrent inflammatory episodes involving various cartilages. The clinical course of RP is variable, and dermatologic manifestations are uncommon. We report a 73-year-old patient who presented with a neutrophilic dermatosis (Sweet's syndrome) as the initial manifestation of RP. There was no evidence for a myelodysplastic syndrome, as it has been previously reported with RP, but the patient was followed-up for an indolent and untreated chronic lymphocytic leukaemia. Complete remission was obtained with oral corticosteroids. This report highlights the clinical spectrum of the RP.     

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.     

Réponse prolongée après traitement par tocilizumab d’une polychondrite atrophiante

Introduction

Relapsing polychondritis (RP) is a rare inflammatory disease characterized by diffuse cartilage involvement, especially those of the respiratory tract, leading to potentially life threatening complications. Corticosteroids remain the first-line empirical therapy. Immunosuppressive drugs such as azathioprine, cyclophosphamide and tumor necrosis factor blockers (anti-TNFα) are commonly used as second-line therapy with varying degrees of success.

Case report

We report a 40-year-old man with severe RP for whom conventional therapy and immunosuppressive treatments were ineffective. Prolonged clinical remission was obtained after introduction of the anti-interleukin-6 receptor antibody (tocilizumab), which was perfectly tolerated and allowed to taper steroids and methotrexate to a very low dosage.

Conclusion

Our patient is the fifth published one documenting the efficacy of tocilizumab in severe refractory RP, which strengthens the use of anti-IL-6 in that indication.     

Manifestations systémiques et auto-immunes des syndromes myélodysplasiques

Myelodysplastic syndromes (MDS) can be associated with systemic or autoimmune diseases. Vasculitides (leucocytoclastic, periarteritis nodosa, micropolyangeitis, Wegener's granulomatosis), relapsing polychondritis, and Sweet's syndrome are the most commonly reported. Refractory anemia with excess of blasts (RAEB), transformed RAEB evolving to an acute leukemia, and chronic myelomonocytic leukemia (CMML) are preferentially associated with these vasculitides or systemic diseases. Corticosteroids are generally effective. Immunosuppressive drugs expose these patients to infectious complications and increase the risk of transformation into acute leukemia. Occurrence of relapsing polychondritis in a patient older than 60 years is associated with a myelodysplastic syndrome in 40% of the cases. Sweet's syndrome is associated in 10% of the cases with acute myeloid leukemia and MDS. Polyarthritis or oligoarthritis with systemic manifestations that include fever, skin rash, and more rarely serositis or haemolytic anemia can occur contemporarily to a MDS. Behçet's disease with intestinal involvement has been reported in patients presenting with trisomy 8 associated MDS. Pathogenic mechanisms underlying the association between MDS and autoimmune or systemic disorders remain to be elucidated.     

Artérite de Takayasu: explorations vasculaires et prise en charge thérapeutique. Expérience à propos de 16 patients

Purpose. -- There is no consensus in regard to vascular explorations and therapeutical management of Takayasu's arteritis. The objective of this study was therefore to establish the most appropriate vascular explorations and to analyze current treatments.Methods. -- Clinical, biological and morphological findings related to either diagnosis or treatment were retrospectively evaluated in sixteen patients diagnosed with Takayasu's arteritis according to the American College of Rheumatology criteria.Results. -- Median delay between the occurrence of the first symptoms and the diagnosis was 9 months. Aortic lesions and aortic valvular incompetence were more frequent. Statistical analysis showed the existence of a correlation between the lack of relapse and corticosteroid therapy (Fisher exact test, P = 0,021). Percutaneous transluminal angioplasty led to stabilization of vascular lesions. Surgical management led to satisfactory results, except for patients with aortic lesions, as survival was then less than 1 year.Conclusion. -- Early diagnosis is mandatory in patients with Takayasu's arteritis in order to propose appropriate therapy, particularly corticosteroid therapy. Surgery and angioplasty prove to be useful in occlusive forms. Late diagnosis is accompanied by severe aortic lesions and fatal outcome.

Résumé

Propos. -- Dans le cadre de l'artérite de Takayasu, il n'existe pas de consensus concernant les explorations vasculaires et la prise en charge thérapeutique. L'objectif de notre étude était d'analyser chez 16 malades souffrant d'artérite de Takayasu les méthodes d'exploration les plus adaptées et les traitements employés.Méthodes. -- Seize patients atteints d'artérite de Takayasu selon les critères de l'American College of Rheumatology ont été évalués de façon rétrospective de 1975 à 1997, cliniquement, biologiquement, morphologiquement, sur le plan diagnostic et thérapeutique.Résultats. -- Dans notre série, la médiane de retard diagnostique était de 9 mois. L'atteinte aortique et valvulaire aortique était plus fréquente. Les résultats ont montré l'existence d'une corrélation statistiquement significative entre l'absence de rechute et l'emploi des corticoïdes (test exact de Fisher, p = 0,021). L'utilisation de l'angioplastie a entraîné une stabilisation des lésions. La prise en charge chirurgicale s'est montrée satisfaisante, en dehors des patients ayant une atteinte aortique pour lesquels la survie était inférieure à 1 an.Conclusion. -- L'analyse de notre série montre la nécessité d'un diagnostic précoce afin de proposer une thérapeutique adéquate, en particulier la corticothérapie. La chirurgie et l'angioplastie sont des recours efficaces en cas de forme occlusive. Les formes diagnostiquées tardivement comportent une atteinte aortique sévère dont l'évolution est le plus souvent fatale.     

Maladie de Lyme avec hépatite et corticothérapie : à propos d’une observation

Introduction

Abnormalities of liver function tests have been occasionally described in large series of Lyme disease, but only one case of hepatitis directly related to infection have been described in literature.

Case report

A 78-year-old-man, with a past medical history of polymyalgia rheumatica (PMR) who had discontinued corticosteroids two years before, presented a transient acute fever and liver cholestasis and cytolysis after an exposure to tick bites. A few days later, cervical pain occurred and corticosteroids were resumed as a PMR relapse was suspected. Hematogenous dissemination with acute meningoradiculitis and multiple erythema migrans led to conclude to a stage 2 Lyme disease.

Conclusion

Although hepatitis complicating the course of Lyme disease has been described in literature, the marked inflammation in our patient led us to investigate the possibility of a co-infection. Also, we discuss the responsibility of corticosteroids in clinical worsening of Lyme disease if they are prescribed without concomitant antibiotics.     

Seuil minimal de réactivité au cours des tests de provocation orale

Determination of the threshold dose for clinical reactivity for a given food is done as a function of certain criteria. For example, the patient must not be aware of the minimal reactivity dose; it should be noted that this dose might vary from time to time. In addition, the food production industry, being concerned with safety issues, would like to know the minimal reactivity dose for specific foods. Currently available data indicate that this dose can be less than 1 mg and, furthermore, that it is difficult to determine with precision.