Unprecedented complexity of six coexisting autoimmune diseases: A case report.

INTRODUCTION: Autoimmune disorders often exhibit interconnectedness, although encountering multiple autoimmune conditions in a single patient is uncommon. Multiple autoimmune syndrome is characterized by the presence of at least three distinct autoimmune diseases in an individual. This report outlines the case of a middle-aged woman diagnosed with autoimmune thyroiditis, Sjögren's syndrome, scleroderma, autoimmune hepatitis, primary biliary cirrhosis, and antisynthetase syndrome. Additionally, it includes a literature review encompassing multiple autoimmune syndromes involving five or more autoimmune diseases. OBSERVATION: A 57-year-old woman, with no previous medical history, presented with fever, extensive muscle weakness, progressive exertional dyspnea, inflammatory polyarthralgia, dysphagia, and dry mouth. Clinical examination revealed muscular deficit in the scapular and pelvic girdles, distal muscular deficit, synovitis in the wrists, and features indicative of "mechanic's hand". Laboratory examinations showed cytolysis, cholestasis, elevated muscle enzymes, hypergammaglobulinemia and elevated thyroid stimulating hormone. Immunoassays showed positive results for antinuclear antibodies, anti-histidyl-t-RNA synthetase, anti-Sjögren's-syndrome-related antigen A, anti-ribonucleic-acid-polymerase-III-RP155, anti-fibrillarin, anti-mitochondrial, anti-liver/kidney microsomal type 1, anti-glycoprotein 210, and anti-thyroid peroxidase antibodies. Further investigations led to the diagnosis of a multiple autoimmune syndrome involving autoimmune thyroiditis, Sjögren's syndrome, scleroderma, autoimmune hepatitis, primary biliary cirrhosis, and antisynthetase syndrome. The patient received treatment with intravenous immunoglobulins, corticosteroids, azathioprine, and ursodeoxycholic acid, which resulted in favorable clinical and biological outcomes. CONCLUSION: This patient presented with six concurrent distinct autoimmune disorders, categorizing this case as a type two multiple autoimmune syndrome. The identification of antisynthetase syndrome notably distinguishes this case.

Epidemio-clinical profile of Takayasu Arteritis at the Center of Tunisia.

INTRODUCTION: Takayasu's Arteritis (TA) is a systemic vasculitis affecting the aorta and its main branches. AIM: To describe the epidemiological, diagnostic, therapeutic and prognostic profile of TA in the referral departments of internal medicine in the Sousse region (Tunisia). METHODS: This is a descriptive, retrospective and exhaustive study, carried out in the two departments of Internal Medicine of Sousse. Patients followed for AT, from 1996 to 2020 were included. The disease was defined according to the classification criteria of the American College of Rheumatology. Disease activity was assessed according to NIH criteria. Age referred to the date of diagnosis. RESULTS: The study population consisted of 40 patients (Sahloul: n=32, Hached: n=8) with a sex ratio=0.17 and a median age=35 years (IIQ=[30-41]). The median diagnostic delay was 5 months (IIQ=[2-14]). The main clinical sign was pulse abolition and/or decrease (78%). Aortic stenosis was the main arterial lesion found (98%). Treatment was based on corticosteroids (95%) and immunosuppressants (42%). The prognosis of TA was often active (62%), with vascular co-morbidity (60%) and iatrogenic complications (35%). CONCLUSION: The epidemiological-clinical profile of AT in the region of Sousse (Tunisia) was characterized by a female predominance, a diagnostic delay, a clinical polymorphism, and evolution towards vascular co-morbidities.

Elderly-onset adult Still's disease.

Adult-onset Still's disease is a rare inflammatory disorder usually affecting young adults. Elderly-onset Still's disease (EOSD) is reported in some cases, commonly in Japan, the USA and Europe. One of the most commonly used criteria for diagnosing EOSD is Yamaguci criteria. In elderly patients more severe course of the disease and more complications may be expected than in the younger group of patients with Still's disease. The lungs involvement is rather rare manifestation of this disease. In our article we discuss the problem of both the development of Still's disease in the elderly and interstitial lung changes in the course of the disease, based on available literature and own cases from one centre.

Takayasu's arteritis occurring under TNF blockers in a patient with spondyloarthritis: is it an association or a paradoxical effect?

Coexistence of spondyloarthritis (SpA) and Takayasu's arteritis is not a common finding, but such cases have been discussed, particularly in the context of choice of therapy. Inhibition of inflammation by tumor necrosis factor inhibitors (TNFi) is a key aspect of the treatment of SpA and also positive effects of such treatment in concomitant large vessel vasculitis have been reported. However, TNFi is also associated with the possibility of initiating vasculitis. The present article based on a case study and the available literature is an attempt to discuss coexistence of these two diseases and the impact of treatment with biological drugs from the anti-TNF group in the course of SpA with Takayasu's arteritis.

Oral Lichen Planus and Lichenoid Lesions in Sjogren's Syndrome Patients: A Prospective Study.

OBJECTIVE: The aim of this study was to investigate the prevalence and characteristics of oral lichen planus (OLP) and oral lichenoid lesions (OLL) in Sjogren's syndrome (SS) patients. PATIENTS AND METHODS: A prospective clinical study was conducted at the Department of Oral Medicine and Oral Surgery in Sahloul Hospital, Sousse, from January 2012 to June 2018. The patients involved in this study were diagnosed with Sjogren's syndrome according to the AECG (American-European consensus group) diagnostic criteria. Among these patients, we searched for those affected by OLP or OLL as determined by the WHO (World Health Organisation) classification of 2003. Clinical variables such as age, sex, medical conditions and medications, type of SS (primary or secondary), clinical form of OLP, and treatment were analyzed. The assessment of the results was performed using SPSS software. RESULTS: We evaluated 30 patients (27 females and 3 males) diagnosed with SS (24 had primary SS) with a mean age of 55 years and 11 months (±11,714). Overall, 9 patients had oral lesions (30%). Two patients had OLP associated with secondary SS (25%). Primary Sjogren's syndrome patients had 6 OLP lesions and one erythematous lichenoid lesion. OLP was erosive in eight patients, among them two had vulvo-vaginal-gingival syndrome. OLP lesions showed improvement in symptoms after topical or general corticosteroids treatment, while OLL showed improvement only under antibiotic treatment. CONCLUSION: The results of our analysis suggest that patients with SS have 30% prevalence of OLP and OLL. This possible association shows the importance of screening for oral dryness in patients with OLP or OLL. Treatment includes topical or general corticosteroids for erosive forms associated or not with topical antifungal treatment to treat or prevent oral candidiasis.