Unusual串行自身免疫

Unusual串行自身免疫

A 46-year-old female patient, known to have rheumatoid arthritis (RA) and secondary Sjögren's syndrome (GSJ), presented with a case of autoimmune myocarditis. This complex condition, part of Multiple Autoimmune Syndrome (MAS) type 3, involves the co-occurrence of three or more autoimmune diseases.

The patient's biological data revealed a markedly elevated troponin level of 1090 ng/ml, indicative of myocardial damage. Cardiovascular disease is well-documented in various autoimmune disorders, making this case particularly concerning.

An MRI revealed abnormalities such as higher T1, T2, and extracellular volume fraction (ECV) values, along with a modest prevalence of late gadolinium enhancement (LGE). These findings are often observed in myocarditis.

The echocardiography showed a nondilated left ventricle with lateral and anterior hypokinesis, further supporting the diagnosis of myocarditis. Notably, the patient's left ventricular ejection fraction (LVEF) was preserved at 59%, but global longitudinal strain was reduced to −14%.

The patient was treated with methotrexate, hydrocortisone, and received adjuvant therapy including folic acid and vitamin D. MTX, classically associated with improved endothelial function and slower atherosclerosis progression, was administered to manage the cardiovascular risks associated with the autoimmune disorders.

The patient's viral serology indicated immunity against cytomegalovirus (CMV), suggesting a possible autoimmune etiology for the myocarditis. Conditions such as autoimmune thyroid disease, RA, myasthenia gravis, aortitis, vitiligo vulgaris, pernicious anemia, inflammatory bowel disease, and orbital myositis have all been associated with GCM.

The co-occurrence of at least 3 autoimmune diseases in a single patient is defined as MAS. The specific pathogenic mechanisms in MAS type 3 arise from intertwined immune dysregulation processes shared among these diseases, characterised by aberrant activation of autoreactive T and B cells, chronic inflammation, and autoantibody production.

Key mechanisms involve dysregulated T cell responses, chronic inflammatory cytokine milieu, skewed T-helper cell responses with reduced immune regulation, and activation of pro-fibrotic pathways. These interconnected mechanisms promote the clinical manifestations of autoimmune myocarditis, rheumatoid arthritis, and Sjögren's syndrome within the syndrome.

Cardiac MRI is considered the test of choice for monitoring cardiotoxicity in patients undergoing long-term treatment with disease-modifying antirheumatic drugs (DMARDs). The patient's case is unique due to the uncommon occurrence of myocarditis in RA, and even more so in Sjögren's syndromes.

This case serves as a reminder of the complexities and challenges in diagnosing and managing autoimmune diseases, particularly when they involve multiple organs. It underscores the importance of regular monitoring and prompt treatment to prevent complications and improve patient outcomes.

1. Ultrasonography is a vital diagnostic tool in monitoring the progression of various medical conditions, including digestive health and eye health.
2. Science continues to unravel the mysteries of chronic diseases, such as autoimmune disorders like rheumatoid arthritis and secondary Sjögren's syndrome, contributing significantly to workplace-wellness.
3. Respiratory conditions, neurological disorders, and skin conditions often intertwine with autoimmune diseases, making diagnosis and treatment a complex challenge.
4. In addition to RA and GSJ, the patient presented with autoimmune myocarditis, a condition associated with Multiple Autoimmune Syndrome (MAS) type 3.
5. MAS type 3, characterized by the co-occurrence of three or more autoimmune diseases, involves intricate immune dysregulation processes.
6. Rheumatoid arthritis, Sjögren's syndrome, and autoimmune myocarditis share aberrant T cell responses, chronic inflammation, and autoantibody production.
7. The patient's elevated troponin level of 1090 ng/ml indicated myocardial damage, a common symptom in cardiovascular disease, well-documented in various autoimmune disorders.
8. The patient's MRI revealed higher T1, T2, and extracellular volume fraction (ECV) values, alongside a modest prevalence of late gadolinium enhancement (LGE), typical findings in MRI imaging of myocarditis.
9. The echocardiography showed nondilated left ventricle with lateral and anterior hypokinesis, supporting the diagnosis of myocarditis and further revealing a preserved left ventricular ejection fraction (LVEF) of 59%.
10. Despite the preserved LVEF, the patient's global longitudinal strain was reduced to −14%, signaling reduced heart muscle function.
11. Methotrexate, hydrocortisone, folic acid, and vitamin D were administered as treatments to manage the cardiovascular risks associated with the patient's autoimmune disorders and to slow atherosclerosis progression.
12. The patient's viral serology tests indicated immunity against cytomegalovirus (CMV), suggesting a possible autoimmune etiology for the myocarditis.
13. Conditions like autoimmune thyroid disease, aortitis, inflammatory bowel disease, and orbital myositis have all been associated with GCM.
14. Cardiac MRI is the preferred test for monitoring cardiotoxicity in patients undergoing long-term treatment with disease-modifying antirheumatic drugs (DMARDs).
15. Regular monitoring is crucial to prevent complications and improve patient outcomes when dealing with autoimmune diseases involving multiple organs.
16. Cardiac MRI is a cornerstone in the advancement of medical-and-wellness, providing critical insights into complex medical conditions.
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