Comparison of Survival Among Patients With Connective Tissue Disease and Cardiomyopathy (Systemic Sclerosis, Systemic Lupus Erythematosus, and Undifferentiated Disease)

doi:10.1016/j.amjcard.2007.03.054

The American Journal of Cardiology

Volume 100, Issue 3, 1 August 2007, Pages 513-517

https://doi.org/10.1016/j.amjcard.2007.03.054 Get rights and content

Connective tissue disease (CTD) encompasses several disease or abnormal states characterized by inflammatory or degenerative changes in connective tissue. These disorders affect many organs, including the heart. However, the long-term survival of patients with cardiomyopathy and CTD is not known. The prognostic implications of different categories of CTD were studied in patients with cardiomyopathy. One thousand seven hundred patients with initially unexplained cardiomyopathy who underwent endomyocardial biopsy from 1983 to 2003 at The Johns Hopkins Hospital were evaluated. Seventy-one subjects were diagnosed with CTD. This cohort was followed for an average of 5.1 years. Among the different disorder categories within CTD, there was a significantly lower survival rate for subjects with undifferentiated CTD (adjusted hazard ratio for death 2.39, 95% confidence interval 1.27 to 4.48, p = 0.007) and a trend toward decreased survival in patients with systemic sclerosis (adjusted hazard ratio for death 1.75, 95% confidence interval 0.93 to 3.29, p = 0.081) compared with those with idiopathic dilated cardiomyopathy (DCM). No significant survival difference was observed between systemic lupus erythematosus and idiopathic DCM (p = 0.41). The long-term outcomes of subjects with CTD and myocarditis were not statistically different from the outcomes of those with CTD and no myocarditis (p = 0.71). In conclusion, the diagnosis of cardiomyopathy and either undifferentiated CTD or systemic sclerosis appears to be a poor prognostic indicator compared with the diagnosis of idiopathic DCM. Patients with systemic lupus erythematosus and cardiomyopathy have a similar prognosis to those with idiopathic DCM. Finally, the presence of myocarditis does not alter the survival of patients with CTD and cardiomyopathy.

Section snippets

Selection of patients

We evaluated 1,700 patients with unexplained cardiomyopathy who underwent endomyocardial biopsy (EMB) from 1983 to 2003 at The Johns Hopkins Hospital. Most of these patients were referred to us by other physicians, and some common causes of heart failure, such as ischemic heart disease, hypertension, and valvular heart disease, were likely underrepresented. At the time of referral to The Johns Hopkins Hospital for the initial evaluation, the causes of patients’ heart failure were unknown.

Clinical and hemodynamic characteristics of the study population

Specific diagnoses for all patients with CTD and cardiomyopathy are listed in Table 1. The 3 most common categories in descending order of frequency were undifferentiated CTD, SLE, and SS. The clinical and hemodynamic characteristics of patients with CTD and those with idiopathic DCM are listed in Table 2. Most patients with CTD were middle aged, white women, with a similar degree of hemodynamic compromise (Table 2). Patients with SLE and cardiomyopathy had significantly higher rates of

Discussion

Our study examined the prognosis of patients with cardiomyopathy and CTD. We explored, for the first time, long-term survival in patients with cardiomyopathy associated with SS, SLE, undifferentiated CTD, or CTD and myocarditis. The finding of a poor outcome in patients with SS relative to those with idiopathic DCM is consistent with previous reports showing a grave prognosis in subjects with SS and clinically significant cardiac involvement12, 17 and may indicate that patients with SS and

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However, myocarditis may have a negative impact on survival [51,66]. Patients with SLE and cardiomyopathy have a similar prognosis to those with idiopathic dilated cardiomyopathy (DCM) [69]. Neonatal cardiac lupus is a rare, passively acquired LM, that encompasses a spectrum of cardiac abnormalities observed in some neonates whose mothers have SLE.
Many systemic immune-mediated diseases (SIDs) may involve the heart and present as myocarditis with different histopathological pictures, i.e. lymphocytic, eosinophilic, granulomatous, and clinical features, ranging from a completely asymptomatic patient to life-threatening cardiogenic shock or arrhythmias. Myocarditis can be part of some SIDs, such as sarcoidosis, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, dermato-polymyositis, eosinophilic granulomatosis with polyangiitis and other vasculitis syndromes, but also of some organ-based immune-mediated diseases with systemic expression, such as chronic inflammatory bowel diseases.
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A 41-year-old female patient with a history of systemic lupus erythematosus presents to the emergency room with symptoms of chest pain. The pain is described as sharp and midsternal, with radiation to the left shoulder. The pain started while at rest and has been constant, although worsens with activity and deep breaths. There is associated shortness of breath but no diaphoresis, nausea, or lightheadedness. No additional symptoms of syncope or edema. The week prior she had experienced symptoms of a cough, malaise, and subjective fevers/chills. The patient was diagnosed with lupus in her late 20s when she presented with profound fatigue and joint pain as well as a malar rash. She is maintained on mycophenolate mofetil and prednisone 5 mg daily. Currently, she is experiencing some increase in the pain in her hands and feet, along with the aforementioned recent chest pain.
Upon presentation to the emergency room, her blood pressure was 112/78, heart rate 82. Her physical exam was remarkable for a malar rash, mild lower extremity edema, and a soft pericardial friction rub. An electrocardiogram was done in the emergency room (Figure 1) and did not show evidence of an acute myocardial infarction. Pertinent labs included a normal white blood cell count, mild anemia, a sedimentation rate of 95 mm/1 h (normal ≤ 29 mm/1 h), and C-reactive protein of 177 mg/L (normal ≤ 9 mg/L). Cardiac biomarkers revealed a troponin T of 0.17 ng/mL at 0 h, 0.19 ng/mL at 3 h, and 0.21 ng/mL at 6 h (normal ≤ 0.01 ng/mL). How would you manage this patient?
Women have an increased risk of certain chronic autoimmune inflammatory disorders (CIDs). All aspects of the heart’s structure and function may be affected by the autoimmune disease. Many of the cardiovascular (CV) complications may be clinically silent and subclinical. However, there is still a clear burden of increased CV morbidity and mortality in these patients. Despite the growing recognition of the increased CV risk in the inflammatory autoimmune patient population, at present there are no specific guidelines that address the risk. A heightened awareness of the CV risk is important as one evaluates and treats the CID patient. Although the CID patient may not report CV symptomatology, their ongoing inflammatory condition may be resulting in damage to the entire cardiac system.
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Most patients are not clinically ill but may die suddenly, presumably secondary to an arrhythmia. Along with a number of cardiac abnormalities (e.g., pericarditis, pericardial effusion, conduction system abnormalities, including complete atrioventricular heart block), DCM can be a rare manifestation of systemic lupus erythematosus and usually correlates with disease activity (169). Studies suggest that echocardiographic evidence of abnormal LV filling may reflect the presence of myocardial fibrosis and could be a marker of subclinical myocardial involvement in systemic lupus erythematosus patients (170).
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CardiomyopathyComparison of Survival Among Patients With Connective Tissue Disease and Cardiomyopathy (Systemic Sclerosis, Systemic Lupus Erythematosus, and Undifferentiated Disease)

Section snippets

Selection of patients

Clinical and hemodynamic characteristics of the study population

Discussion

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

J Chron Dis

J Am Coll Cardiol

Rheum Dis Clin North Am

Rheum Dis Clin North Am

J Vasc Surg

Systemic lupus erythematosus-survival patterns: experience with 609 patients

JAMA

The heart in polymyositis-dermatomyositis

J Neurol

Immunologic studies in patients with Takayasu’s arteritisIII. Immunoregulatory changes

Clin Exp Rheumatol

Cardiomyopathy
Comparison of Survival Among Patients With Connective Tissue Disease and Cardiomyopathy (Systemic Sclerosis, Systemic Lupus Erythematosus, and Undifferentiated Disease)