Multiple Autoimmune Syndrome: An Unusual Combination of Autoimmune Disorders

Author(s): Sabahat Sarfaraz*, Sabiha Anis

Journal Name: Reviews on Recent Clinical Trials

Volume 15 , Issue 3 , 2020


Become EABM
Become Reviewer
Call for Editor

Graphical Abstract:


Abstract:

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.

Keywords: Celiac disease, CREST syndrome, multiple autoimmune syndrome, primary biliary cholangitis (PBC), antinuclear antibodies (ANA), anti-centromere pattern.

[1]
Latif S, Jamal A, Memon I, Yasmeen S, Tresa V, Shaikh S. Multiple autoimmune syndrome: Hashimoto’s thyroiditis, coeliac disease and Systemic Lupus Erythematosus (SLE). J Pak Med Assoc 2010; 60(10): 863-5.
[PMID: 21381622]
[2]
Mohan MP, Ramesh TC. Multiple autoimmune syndrome. Indian J Dermatol Venereol Leprol 2003; 69(4): 298-9.
[PMID: 17642919]
[3]
Cojocaru M, Cojocaru IM, Silosi I. Multiple autoimmune syndrome. Maedica (Buchar) 2010; 5(2): 132-4.
[PMID: 21977137]
[4]
Sloka S. Observations on recent studies showing increased co-occurrence of autoimmune diseases. J Autoimmun 2002; 18(3): 251-7.
[http://dx.doi.org/10.1006/jaut.2002.0588] [PMID: 12126638]
[5]
Rojas-Villarraga A, Amaya-Amaya J, Rodriguez-Rodriguez A, Mantilla RD, Anaya JM. Introducing polyautoimmunity: Secondary autoimmune diseases no longer exist. Autoimmune Dis 2012; 2012: 254319.
[http://dx.doi.org/10.1155/2012/254319] [PMID: 22454759]
[6]
Boccuti V, Perrone A, D’Introno A, Campobasso A, Sangineto M, Sabbà C. An unusual association of three autoimmune disorders: Celiac disease, systemic lupus erythematosus and Hashimoto’s thyroiditis. Auto Immun Highlights 2016; 7(1): 7.
[http://dx.doi.org/10.1007/s13317-016-0079-9] [PMID: 27383232]
[7]
Harpreet S, Deepak J, Kiran B. Multiple autoimmune syndrome with celiac disease. Reumatologia 2016; 54(6): 326-9.
[http://dx.doi.org/10.5114/reum.2016.64911] [PMID: 28115785]
[8]
Lauret E, Rodrigo L. Celiac disease and autoimmune-associated conditions. BioMed Res Int 2013; 2013: 127589.
[http://dx.doi.org/10.1155/2013/127589] [PMID: 23984314]
[9]
Kitic I, Boskovic A, Stankovic I, Prokic D. Twelve-year-old girl with primary biliary cirrhosis. Case Rep Pediatr 2012; 2012: 937150.
[http://dx.doi.org/10.1155/2012/937150] [PMID: 23243543]
[10]
Anaya JM. The autoimmune tautology. A summary of evidence. Joint Bone Spine 2017; 84(3): 251-3.
[http://dx.doi.org/10.1016/j.jbspin.2016.11.012] [PMID: 28017820]
[11]
Cárdenas-Roldán J, Rojas-Villarraga A, Anaya JM. How do autoimmune diseases cluster in families? A systematic review and meta-analysis. BMC Med 2013; 11: 73.
[http://dx.doi.org/10.1186/1741-7015-11-73] [PMID: 23497011]
[12]
Kaukinen K, Collin P, Mykkänen AH, Partanen J, Mäki M, Salmi J. Celiac disease and autoimmune endocrinologic disorders. Dig Dis Sci 1999; 44(7): 1428-33.
[http://dx.doi.org/10.1023/A:1026612121041] [PMID: 10489930]
[13]
Spadaccino AC, Basso D, Chiarelli S, et al. Celiac disease in North Italian patients with autoimmune thyroid diseases. Autoimmunity 2008; 41(1): 116-21.
[http://dx.doi.org/10.1080/08916930701620209] [PMID: 18176874]
[14]
Lidén M, Kristjánsson G, Valtýsdóttir S, Hällgren R. Gluten sensitivity in patients with primary Sjögren’s syndrome. Scand J Gastroenterol 2007; 42(8): 962-7.
[http://dx.doi.org/10.1080/00365520701195345] [PMID: 17613926]
[15]
Rigamonti C, Bogdanos DP, Mytilinaiou MG, Smyk DS, Rigopoulou EI, Burroughs AK. Primary biliary cirrhosis associated with systemic sclerosis: diagnostic and clinical challenges. Int J Rheumatol 2011; 2011: 976427.
[http://dx.doi.org/10.1155/2011/976427] [PMID: 22187566]
[16]
Milkiewicz M, Caballería L, Smyk DS, Milkiewicz P. Predicting and preventing autoimmunity: The case of anti-mitochondrial antibodies. Auto Immun Highlights 2012; 3(3): 105-12.
[http://dx.doi.org/10.1007/s13317-012-0038-z] [PMID: 26000133]


Rights & PermissionsPrintExport Cite as

Article Details

VOLUME: 15
ISSUE: 3
Year: 2020
Published on: 21 June, 2020
Page: [240 - 243]
Pages: 4
DOI: 10.2174/1574887115666200621184110
Price: $65

Article Metrics

PDF: 22
HTML: 3