Type 3 multiple autoimmune syndrome: A case report

Background: Autoimmune diseases are multifactorial with environmental and heritable factors. Autoimmunity reflects an altered immune status, therefore the presence of more than one disorder is not uncommon. The coexistence of three or more autoimmune diseases in a patient constitutes multiple autoimmune syndrome (MAS). This is an interesting case of a middle-aged female who had celiac disease, primary biliary cholangitis, autoimmune hepatitis and evolving CREST (Calcinosis, Rhaynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasia) syndrome. Case Report: Fifty years old female patient presented with generalized fatigue, fever, weight loss, vertigo and constipation. She was a diagnosed case of celiac disease, and responded well to glutenfree diet. Family history was unremarkable for any autoimmune disorder. Laboratory workup showed normal complete blood counts, markedly elevated transaminases and alkaline phosphates. Her antinuclear antibodies (ANA) test was strongly positive (>1:320) and showed an anti-centromere pattern. Anti-extractable nuclear antibody(ENA) assay showed anti-mitochondrial and anti- CENP B antibodies. Liver biopsy revealed overlap syndrome (primary biliary cholangitis and autoimmune hepatitis). : This patient had celiac disease, primary biliary cholangitis and autoimmune hepatitis. Extensive immunological workup unexpectedly revealed the presence of anti-centromere protein B (anti-CENP B) antibodies which are strongly associated with CREST syndrome. Clinical re-evaluation of the patient gave clues of the evolving CREST syndrome. This case report highlights the importance of adequate immunological investigations in conjunction with clinical information for adequate patient management to achieve favorable consequences in the future. Conclusion: Patients suffering from an autoimmune disease need special attention as multiple immune- mediated disorders may be present simultaneously or sequentially during the course of the disease process. MAS patients are at a higher risk of acquiring infections and tumor development due to prolonged use of immunosuppressants. These patients need close surveillance for the development of another autoimmune disease, so as to control the current disease and to prevent future complications. This case report emphasizes the importance of a multidisciplinary team approach including an immunologist who may facilitate a better understanding of disorders related to the breakdown of immune tolerance.

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AB0776 MULTIPLE AUTOIMMUNE SYNDROME

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3297 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1415.1-1415

Author(s):

A. Fraj ◽

S. Arfa ◽

O. Jomaa ◽

F. Boubaker ◽

I. Bannour ◽

...

Keyword(s):

Internal Medicine ◽

Autoimmune Diseases ◽

Clinical Signs ◽

Primary Biliary Cholangitis ◽

Medicine Department ◽

Internal Medicine Department ◽

Autoimmune Syndrome ◽

Multiple Autoimmune Syndrome ◽

Type 3

Background:Multiple autoimmune syndrome (MAS) is a rare entity, defined by the association of three autoimmune diseases in the same patient. MAS can be classified into three groups.Objectives:The objective of this work was to describe the autoimmune diseases profile in MAS in An Internal Medicine Department.Methods:We report a retrospective analysis including 14 cases of MAS seen in The Internal Medicine Department at Taher Sfar Hospital, Mahdia, TUNISIA over a period of 10 years.Results:We followed 14 patients with MAS. They were 14 women. The mean age of patients was 52 years. SAM was type 3 in 12 patients (85%), type 2 in one patient (7.1%) and one patient satisfied both type 2 and type 3 MAS criteria (7.1%). No cases of MAS type 1 had been reported. We found 13 patients with 3 associated autoimmune diseases and one patient with 4 associated autoimmune diseases. The autoimmune diseases were: Sjögren’s syndrome in 14 patients (100%), Hashimoto’s thyroiditis in 11 cases (78%), systemic lupus erythematosus in 7 cases (50%), Addison’s disease in 4 cases (28, 5%), scleroderma, thymoma, vitiligo, Biermer and primary biliary cholangitis each in one case (7.1%).Conclusion:Multiple autoimmune syndrome remains a rare but probably under-diagnosed entity. Thus, in patients with autoimmune disease, initial investigation and follow-up of clinical signs and biological stigmas of other autoimmune diseases should be cautious.Disclosure of Interests:None declared

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Cocaine/Levamisole-Associated Autoimmune Syndrome: A Case Report

Actas Dermo-Sifiliográficas (English Edition) ◽

10.1016/j.adengl.2021.07.004 ◽

2021 ◽

Author(s):

M.I. Cevallos Abad ◽

A. Córdoba Iturriagagoitia ◽

M. Larrea García

Keyword(s):

Case Report ◽

Autoimmune Syndrome

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Differential diagnosis of a thyroid mass, facial malar rash and ptosis on the flora in the primavera by Sandro Botticelli (1445–1510)

Journal of Endocrinological Investigation ◽

10.1007/s40618-021-01623-3 ◽

2021 ◽

Author(s):

H. Ashrafian

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Systemic Lupus ◽

Bilateral Ptosis ◽

Malar Rash ◽

Autoimmune Syndrome ◽

Thyroid Mass ◽

Multiple Autoimmune Syndrome ◽

Sandro Botticelli ◽

Renaissance Artist

Abstract Purpose The Primavera is considered amongst the greatest and controversial artistic masterpieces worldwide painted by renaissance artist Sandro Botticelli. The aim was to identify any underlying medical foundations for the painting. Methods Observational study. Results The painting reveals, a ‘butterfly’ malar rash, bilateral ptosis and a clear neck swelling consistent with a goitre in the figure of Flora. This could be explained by concomitant Graves’ disease and systemic lupus erythematosus, or other presentations of multiple autoimmune syndrome. Conclusion These findings highlight the likely presentation of the earliest pictorial depictions of thyroid disease with systemic lupus erythematosus and emphasize the exactitude of depiction demonstrated by Botticelli in renaissance era.

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