结节病易误诊,据王洪武等~([1])收集国内18篇关于此病误诊的文献,误诊率高达63.2%,当然有误诊就会有误治,如孙永昌等~([2])报道26例结节病在影像学检查诊断的第一印象中拟诊结核5例,其中就有2例完成规范的抗结核治疗,为此应引起临床对本病诊治的重视。

Sarcoidosis

Objectives: To develop evidence-based European League Against Rheumatism (EULAR) recommendations for the management of Behcet disease (BD) supplemented where necessary by expert opinion. Methods: The multidisciplinary expert committee, a task force of the EULAR Standing Committee for Clinical Affairs (ESCCA), consisted of nine rheumatologists (one who was also a clinical epidemiologist and one also a Rehabilitation Medicine doctor), three ophthalmologists, one internist, one dermatologist and one neurologist, representing six European countries plus Tunisia and Korea. A patient representative was also present. Problem areas and related keywords for systematic literature research were identified. Systematic literature research was performed using Medline and the Cochrane Library databases from 1966 through to December 2006. A total of 40 initial statements were generated based on the systematic literature research. These yielded the final recommendations devel- oped from two blind Delphi rounds of voting. Results: Nine recommendations were developed for the management of different aspects of BD. The strength of each recommendation was determined by the level of evidence and the experts' opinions. The level of agreement for each recommendation was determined using a visual analogue scale for the whole committee and for each individual aspect by the subgroups, who consider themselves experts in that field of BD. There was excellent concordance between the level of agreement of the whole group and the ''experts in the field''. Conclusion: Recommendations related to the eye, skin- mucosa disease and arthritis are mainly evidence based, but recommendations on vascular disease, neurological and gastrointestinal involvement are based largely on expert opinion and uncontrolled evidence from open trials and observational studies. The need for further properly designed controlled clinical trials is apparent.

https://ard.bmj.com/content/annrheumdis/67/12/1656.full.pdf

EULAR recommendations for the management of Behçet disease

Kynurenine pathway in major depression: evidence of impaired neuroprotection.

The neurodegeneration hypothesis proposed major depression as a consequence of the imbalance between neuroprotective and neurodegenerative metabolites in the kynurenine pathway. To test the hypothesis, plasma tryptophan and kynurenine pathway metabolites were studied in 58 patients with major depression and 189 normal controls. The mean tryptophan breakdown index was higher (p=0.036), and mean kynurenic acid concentration and mean neuroprotective ratios were lower, in depressed patients (p=0.003 and 0.003, respectively). In receiver operating characteristic analysis, the kynurenic acid concentrations and the neuroprotective ratio showed clear discrimination between depressed patients and controls with area under the curve 79% and 76.3% respectively. The neuroprotective ratio did not change after treatment in those with repeated episodes of depression but it increased significantly (p=0.044) in those with first episodes. The results suggested that the reduction in neuroprotective markers, which indicated an impaired neuroprotection, might play an important role in pathophysiology of major depression.

Research report Kynurenine pathway in major depression: Evidence of impaired neuroprotection

Current Concepts in the Etiology and Treatment of Behçet Disease

Bullous pemphigoid (BP) is an autoimmune disorder characterized by the production of autoantibodies against two antigens (BPAG1 and 2) and can be induced by drugs (1,2). Recently, the development of BP was reported in diabetic patients treated with dipeptidyl peptidase-4 inhibitors (gliptins) plus metformin (3). However, it remains unknown whether gliptins alone or in combination with metformin are responsible for the induction of BP. We report two diabetic patients who developed BP shortly after addition of vildagliptin to their metformin monotherapy.

A 59-year-old woman and a 67-year- old man, both with type 2 diabetes that had been controlled with metformin for 6 and 3 years, respectively, presented with an 1- and 3-month history of pruritic …

https://care.diabetesjournals.org/content/diacare/34/8/e133.full.pdf

Dipeptidyl Peptidase-4 Inhibitors Cause Bullous Pemphigoid in Diabetic Patients: Report of Two Cases

Georgiou S, Monastirli A, Pasmatzi E, Gartaganis S, Goerz G, Tsambaos D (Departments of Dermatology and Ophthalmology, University of Patras, Greece and the Department of Dermatology, Heinrich-Heine University of Dusseldorf, Germany). Efficacy and safety of systemic recombinant interferon-alpha in Behcet's disease. J Intern Med 1998; 243: 367–72.



Objectives

To evaluate the therapeutic efficacy and safety of systemic recombinant interferon α-2a (IFN -α) in patients with Behcet's disease (BD) and to determine the incidence of episodes in complete responders during the one-year pretreatment period and follow-up.



Design

An open clinical study.



Setting

Departments of Dermatology and Ophthalmology, University of Patras, Greece and Department of Dermatology, Heinrich-Heine University of Dusseldorf, Germany.



Subjects

Twelve patients (aged 23–52 years) with active BD who had previously been unsuccessfully treated with systemic steroids and/or immunosuppressives.



Interventions

IFN-α was administered subcutaneously at a dose of 6 × 106 IU per day 3 times per week for 2 months.



Main outcome measures

Change of area or number of mucocutaneous lesions, grading score for thrombophlebitis and ocular inflammation, haematological and biochemical parameters and number of episodes during the pretreatment period and the follow-up. Evaluation of IFN-α side effects.



Results

Nine patients (75.0%) revealed a complete remission, two (16.6%) a partial remission and one patient (8.3%) showed no response. During the follow-up in five out of the nine complete responders (55.5%) no episodes of BD were seen, whereas, the other four patients (44.5%) had 1–2 episodes, as compared to 5–8 and 5–12 episodes, respectively, during the pretreatment period. An influenza-like syndrome (fever, nausea and myalgias) appeared during the early phase of therapy in all (but one) patients. No patient had to discontinue IFN-α because of intolerance.



Conclusions

Subcutaneous human recombinant interferon α-2a appears to be an effective and fairly well tolerated therapy for BD.

Efficacy and safety of systemic recombinant interferon‐alpha in Behcet's disease

Although neuromuscular adverse effects represent significant clinical manifestations of hypervitaminosis A syndrome, surprisingly little attention has been paid to the potential neuromuscular toxicity of vitamin A derivatives (retinoids). Since isotretinoin and acitretin are currently the two most commonly used oral retinoids in systemic dermatotherapy, this review focuses exclusively on their neuromuscular adverse effects and proposes a neuromuscular algorithm for appropriate monitoring of patients treated with these two compounds. The most frequent CNS adverse effect associated with oral isotretinoin is headache, either as an independent adverse effect or as part of benign intracranial hypertension, which is additionally characterized by nausea and visual changes. Isolated cases of stiff-person-like syndrome, epileptic seizures and generalized muscle stiffness syndrome, possibly or probably related to oral treatment with isotretinoin, have also been reported. In addition, oral isotretinoin has reportedly been associated with muscular adverse effects that most frequently manifest as myalgia and stiffness and, in rare cases, as true myopathy or rhabdomyolysis. Creatine phosphokinase, a specific marker of muscle destruction, has been found to be elevated, occasionally by up to 100 times the normal value (with or without muscular symptoms and signs), in a variable percentage of patients receiving isotretinoin treatment and particularly in those undergoing vigorous physical exercise. Oral acitretin has been found to cause peripheral nerve dysfunction, particularly of sensory fibres, which in rare cases leads to clinically evident sensory disturbances. Less clear is the causal relationship between acitretin and benign intracranial hypertension or myopathy, whereas an isolated case of cranial nerve IV (oculomotor) palsy and a further case of thrombotic stroke during treatment with oral acitretin have been reported. Systemic diseases with involvement of nervous and/or muscle tissue and neuromuscular disorders should be regarded as exclusion criteria for initiation of oral retinoid therapy. Additionally, intense physical exercise and concurrent treatment with neurotoxic or myotoxic drugs should be avoided during treatment with oral retinoids. In order to minimize the potential risk of neuromuscular adverse effects, a neuromuscular algorithm is suggested that may be useful for monitoring patients taking oral retinoids.

Neuromuscular adverse effects associated with systemic retinoid dermatotherapy: monitoring and treatment algorithm for clinicians

Major depression and risk of depressive symptomatology associated with short-term and low-dose interferon-α treatment

We report a 31-year-old female patient with cutaneous sarcoidosis, who showed a complete remission of her single system skin disease after an 8-month therapy with oral isotretinoin (1 mg/kg/day). At 15-month follow-up, the patient still remained free of recurrence and visceral involvement.

Alexandra Monastirli

Papers

Dipeptidyl Peptidase-4 Inhibitors Cause Bullous Pemphigoid in Diabetic Patients: Report of Two Cases

Efficacy and safety of systemic recombinant interferon‐alpha in Behcet's disease

Neuromuscular adverse effects associated with systemic retinoid dermatotherapy: monitoring and treatment algorithm for clinicians

Major depression and risk of depressive symptomatology associated with short-term and low-dose interferon-α treatment

Cutaneous sarcoidosis: complete remission after oral isotretinoin therapy.