Autoinflammatory Disorders

The aetiology of Crohn’s disease and paratuberculosis are the subjects of intensive study and also frequently, of dispute. However, a number of other nosological entities have a similar history, namely type 1 diabetes, multiple sclerosis, sarcoidosis, asthma, psoriasis, spondylarthritis, Blau syndrom etc. The zoonotic risk of Mycobacterium avium subsp. paratuberculosis (MAP) has been discussed for more than one hundred years. „The problem remains open, further research is needed“, is the sentence which seems to be obligatory in the conclusions
of many scientific articles. A number of hypotheses have been suggested, all with a grain of truth in them. The infection hypothesis has many supporters and opponents, but it does not fit to all Crohn’s disease cases. The contribution of the genetic factor has been admitted a long time ago and has been experimentally confirmed by recent excellent studies. An environmental factor is expected and has been often mentioned, but has yet to be discovered. Muramyl dipeptide, derived from peptidoglycans of the bacterial cell wall is one of the triggers, mentioned in connection with chronic inflammatory diseases. The immunomodulatory ability of this compound has been recognised for decades and is exploited in Freund’s adjuvant. A critical amount of muramyl dipeptide can
affect immunity during some bacterial infections but the long latent period between infection and onset of the clinical form of the disease could explain why a causative relationship between the primary infection and chronic inflammation is not considered. Different species of mycobacteria can be found in the environment, in water, dust, soil and aerosol. Although severe infections with mycobacteria have been described, these species are not thought to be typical zoonotic pathogens. Muramyl dipeptide derived from mycobacteria obviously plays a starring role as a bacterial trigger in the aetiology of many autoimmune and autoinflammatory diseases. Paratuberculosis in cattle and other ruminants is a source of enormous contamination of the environment but also of milk and meat by MAP. Muramyl dipeptide from mycobacteria, namely MAP, and Crohn’s disease as a representative of diseases often called civilization threats, are important pieces of the gigantic puzzle. Mycobacteria in the environment and foodstuffs have to be acknowledged as a public health risk, which can never be completely eliminated. There is no reason to push the panic button, but we must learn how to live  together with this microorganism, how the pool of immunomodulator sources can be diminished, and how the pathogenic relationship between triggers and target tissues can be disrupted. The dissemination of knowledge, the availability of rapid and inexpensive tools for identification of mycobacteria in different matrices, and the establishment of a maximal allowed limit for mycobacteria in milk and meat should contribute to food safety and consumer protection.

Behcet's disease (BD) is a rare condition, considered as a variable vessel vasculitis with multi-system involvement, characterized by the triad of recurrent mouth and genital ulcers with eye involvement, also may involve joints, skin, central nervous system, and gastrointestinal tract with important heterogeneity among patients regarding demographic features and organ manifestations. BD has a worldwide distribution. However, it is observed commonly among populations living along the historic Silk Road.The etiology of the disease remains unknown, but the most widely held hypothesis of disease pathogenesis is that of a profound inflammatory response triggered by an infectious agent in a genetically susceptible host. Number of reported cases is inexplicably low in sub-Saharan region and especially lowest in West Africa where only few cases have been reported. The aim of this presentation is to announce the first case description of BD in Gambia as well as, to sensitize physicians, Internal Medicine residents and general practitioners in our sub region with the pattern of the disease, as only way to reduce the diagnosis time frame.

Abstract
Objective: to report a typical case of Periodic fever associated with aphthous stomatitis, pharyngitis and adenitis (PFAPA); review its characteristics and how to diagnose it in an emergency service.
Case report: female patient, 1 year and 5 months old, presented with recurrent episodes of fever, irritability and lesions of the oral mucosa. The symptoms last for a week and occur monthly since the patient was five months old. Other diseases were excluded and the patient was treated with prednisone during the episodes with improvement of symptoms.
Conclusion: there are several auto-inflammatory diseases described, but the PFAPA is distinguished not only for being the most frequent, but also for being sporadic and for benign nature. The PFAPA is a relatively newly described syndrome that requires dissemination in academic circles leading to a higher index of suspicion among physicians and pediatricians, especially those who take care of patients in the emergency room avoiding unnecessary treatments and evaluation.
Key words: periodic fever, Auto-inflammatory diseases, stomatitis, pharyngitis, lymphadenopathy