Unusual Multiple Autoimmune Disorder Case: A Case Report Highlighting Myocarditis
A 46-year-old female with known autoimmune disorders, specifically rheumatoid arthritis (RA) and secondary Sjogren's syndrome (GSJ), was admitted to the cardiac intensive care unit after presenting with acute chest pain, chronic fever, and swelling of the left wrist.
The patient's cardiac MRI confirmed the diagnosis of myocarditis, a condition that can be a manifestation of systemic autoimmune diseases. In this case, it appeared to be of autoimmune origin, combining the three conditions in an atypical presentation.
The MRI revealed T1, T2, and extracellular volume fraction (ECV) values higher than those in controls, with a modest prevalence of late gadolinium enhancement (LGE) at 18%. The MRI signs of rheumatoid myocarditis have not been specifically reported, but similar findings are observed in other autoimmune rheumatic diseases (AIRD), such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and inflammatory myopathies.
Myocarditis is classified as type 3 MAS and typically does not present with connective tissue involvement. Type 3 MAS commonly involves the coexistence of several autoimmune diseases, predominantly autoimmune thyroid disease, type 1 diabetes mellitus (T1DM), and another organ-specific autoimmune disorder. In this case, the patient's biological data revealed a markedly elevated troponin level of 1090 ng/ml, suggesting myocardial damage.
The patient was treated with 20 mg methotrexate weekly and had a history of corticosteroid-induced adrenal insufficiency, for which she was administered 20 mg hydrocortisone daily. The guideline-based therapy for patients with acute myocarditis is supportive therapy for left ventricular dysfunction.
Myocardial biopsy is typically reserved for cases of fulminant myocarditis that do not improve after a few days or when a nonlymphocytic cause is suspected. However, in this case, the MRI findings were conclusive, and a biopsy was not performed.
Cardiac MRI is considered the test of choice for monitoring cardiotoxicity in patients undergoing long-term treatment with disease-modifying antirheumatic drugs (DMARDs), such as methotrexate (MTX). MTX is classically associated with improved endothelial function, slower atherosclerosis progression, decreased risk of major cardiovascular adverse events, and benefits on cardiovascular survival.
The patient was discharged after a 3-day stay with a favorable clinical progression. This case highlights the importance of considering myocarditis as a potential complication in patients with autoimmune rheumatic diseases and the role of cardiac MRI in its diagnosis and monitoring.
Summary Table
| Condition | Association with Type 3 MAS | Relation to Myocarditis | |-----------------------------|----------------------------------------|--------------------------------------------------| | Autoimmune thyroid diseases | Core component | No direct myocarditis; but may co-occur with other autoimmune diseases causing myocarditis | | Type 1 Diabetes Mellitus (T1DM) | Core component | Usually no myocarditis; primarily pancreatic | | Systemic Lupus Erythematosus (SLE) | Often coexists in MAS or type 3 overlap | Can cause myocarditis as part of systemic involvement | | Mixed Connective Tissue Disease (MCTD) | Possible overlap condition | Can cause myocarditis via systemic inflammation |
Key Points
- Type 3 MAS refers to multiple coexisting autoimmune conditions, mostly including thyroid autoimmunity plus others like T1DM or systemic autoimmune diseases.
- Myocarditis is an autoimmune inflammation of the heart muscle that occurs secondary to systemic autoimmune diseases such as SLE, which may be components of type 3 MAS.
- Therefore, myocarditis fits into the type 3 MAS context as a complication or organ involvement rather than a primary autoimmune disease in MAS.
- The patient presented with MRI-confirmed myocarditis, likely of autoimmune origin, combining the 3 conditions in an atypical presentation.
- Cardiac MRI is considered the test of choice for monitoring cardiotoxicity in patients undergoing long-term treatment with DMARDs.
- MTX (methotrexate) is classically associated with improved endothelial function, slower atherosclerosis progression, decreased risk of major cardiovascular adverse events, and benefits on cardiovascular survival.
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