**8. Management**

The medical and interventional management in sarcoidosis requires interaction of a multidisciplinary team that includes a cardiologist, electrophysiologist, rheumatologist, pneumologist, and other specialists.

**117**

therapy [26, 27].

immunosuppression therapy.

*Indication of immunosuppression therapy.*

*Cardiac Sarcoidosis*

*DOI: http://dx.doi.org/10.5772/intechopen.85310*

**Histological diagnosis of myocardial tissue**

identified cause (including staining for microorganisms)

a. Histological diagnosis of extracardiac sarcoidosis

sistent with cardiac sarcoidosis)

b. One or more of the following:

sarcoidosis)

*Diagnostic criteria for sarcoidosis (HRS) 2014.*

**Table 2.**

**Table 3.**

inflammation

**Clinical diagnosis (invasive studies and noninvasive): recognized as probable**

All patients with cardiac sarcoidosis should have a proper control of cardiovascular risk factors, management of cardiac failure in case it occurs, treatment of ventricular arrhythmias and atrioventricular conduction malfunction, as well as

Non-sustained ventricular arrhythmia and frequent ventricular ectopy and evidence of myocardial

Presence of granuloma not classified in the histological exam of the myocardial tissue without an alternative

I.Cardiac block or cardiomyopathy with response to immunosuppression therapy with steroids

V.Irregular captation in a positron emission heart tomography (a consistent pattern with cardiac

VI. Delayed enhancement with gadolinium in a cardiac nuclear magnetic resonance (in a pattern con-

II. Ejection fraction of the left ventricle reduced in an unexplainable form (<40%) III. Sustained ventricular tachycardia with no clear cause (spontaneous or induced) IV. Atrioventricular block second-degree Mobitz II or cardiac block third degree

VII. Positive gallium captation (in a pattern consistent with cardiac sarcoidosis)

c. Other causes of the cardiac manifestations have been reasonably excluded

Left ventricular dysfunction and evidence of myocardial inflammation

Cardiac block Mobitz II and degree III and evidence of myocardial inflammation Sustained ventricular arrhythmia and evidence of myocardial inflammation

Immunosuppression therapy is prescribed in all patients with cardiac sarcoidosis. The criteria shown in **Table 3** should be fulfilled [27]. Corticosteroids (prednisolone goes from 30 to 40 mg/day) [32] and the clinical response should be assessed 1–3 months after the starting treatment. The dosage should be reduced gradually, as low as 5–15 mg/day, until completing 9–12 months of sustained treatment. The patients should undergo clinical follow-up for up to 3 years after the therapy to identify relapse. Other therapies (second or third line) include methotrexate, infliximab, cyclophosphamide, and azathioprine, used in refractory cases or when steroids adverse effects are not tolerated. In immunosuppression treatment it is essential to identify the inflamed myocardium via histological study or imaging. It is here where fluorodeoxyglucose positron 18 emission tomography becomes important. It is worth noting this is proposed as a follow-up strategy to define which patients should continue with or discontinue

**Table 4** lists the recommendations by consensus for the management of

arrhythmias related to cardiac sarcoidosis [4, 27].

#### **Histological diagnosis of myocardial tissue**

Presence of granuloma not classified in the histological exam of the myocardial tissue without an alternative identified cause (including staining for microorganisms)

#### **Clinical diagnosis (invasive studies and noninvasive): recognized as probable**

a. Histological diagnosis of extracardiac sarcoidosis

	- I.Cardiac block or cardiomyopathy with response to immunosuppression therapy with steroids
	- II. Ejection fraction of the left ventricle reduced in an unexplainable form (<40%)
	- III. Sustained ventricular tachycardia with no clear cause (spontaneous or induced)
	- IV. Atrioventricular block second-degree Mobitz II or cardiac block third degree
	- V.Irregular captation in a positron emission heart tomography (a consistent pattern with cardiac sarcoidosis)
	- VI. Delayed enhancement with gadolinium in a cardiac nuclear magnetic resonance (in a pattern consistent with cardiac sarcoidosis)
	- VII. Positive gallium captation (in a pattern consistent with cardiac sarcoidosis)

c. Other causes of the cardiac manifestations have been reasonably excluded

#### **Table 2.**

*Sarcoidosis and Granulomatosis - Diagnosis and Management*

Patients without extracardiac compromise

than 60 years old without an evident cause

**5.5 Biomarkers**

**5.6 Endomyocardial biopsy**

**6. Diagnostic criteria**

shown in **Table 2** [27].

**8. Management**

**7. Differential diagnostic**

• Monomorphic ventricular tachycardia without a clear cause

more studies are needed to assess its use in the clinical setting.

the multifocal nature of the infiltration [30, 31].

matologist, pneumologist, and other specialists.

• One or more of the following echocardiographic anomalies: anomalies in the regional movement of a wall, ventricular aneurysm, thinning of the interventricular septum (basal segment), and ejection fraction of the left ventricle below 50%

• Atrioventricular block second-degree Mobitz II or third degree in adults younger

The angiotensin-converting enzyme has been found to be high in 75% of the patients with non-treated sarcoidosis, it does not have much diagnostic value due to its low sensitivity and specificity, and multiple conditions such as diabetes mellitus, tuberculosis, hyperthyroidism, and lung cancer, among other entities, can alter its levels [28]. The measurement of the soluble receptor of interleukin 2 has been proposed as an inflammatory marker in patients with extrapulmonary disease [28];

Being an invasive procedure and considering that sarcoidosis is usually a disease with multisystem compromise, it is preferred to identify a possible extracardiac site for biopsy and histological studies (lymph nodes or lungs). In cases in which the histological tests are not conclusive or there is only cardiac compromise, the endomyocardial biopsy becomes important in the diagnosis of this condition. The diagnostic performance of a blind biopsy is 25% [29] increasing to 50% when guided by images or electroanatomic mapping; this is logical when taking into consideration

There is no global consensus accepted for the diagnosis of cardiac sarcoidosis. In 2014 The European Heart Rate Society (HRS) published a consensus for the histological and clinical diagnosis of sarcoidosis with cardiac compromise which is

The differential diagnosis of this condition is difficult considering all the pathologies that can manifest in a similar form. The complete medical record and the physical exam are the most important ways to make a diagnostic approach.

The medical and interventional management in sarcoidosis requires interaction of a multidisciplinary team that includes a cardiologist, electrophysiologist, rheu-

**116**

*Diagnostic criteria for sarcoidosis (HRS) 2014.*


#### **Table 3.**

*Indication of immunosuppression therapy.*

All patients with cardiac sarcoidosis should have a proper control of cardiovascular risk factors, management of cardiac failure in case it occurs, treatment of ventricular arrhythmias and atrioventricular conduction malfunction, as well as immunosuppression therapy.

Immunosuppression therapy is prescribed in all patients with cardiac sarcoidosis. The criteria shown in **Table 3** should be fulfilled [27]. Corticosteroids (prednisolone goes from 30 to 40 mg/day) [32] and the clinical response should be assessed 1–3 months after the starting treatment. The dosage should be reduced gradually, as low as 5–15 mg/day, until completing 9–12 months of sustained treatment. The patients should undergo clinical follow-up for up to 3 years after the therapy to identify relapse. Other therapies (second or third line) include methotrexate, infliximab, cyclophosphamide, and azathioprine, used in refractory cases or when steroids adverse effects are not tolerated. In immunosuppression treatment it is essential to identify the inflamed myocardium via histological study or imaging. It is here where fluorodeoxyglucose positron 18 emission tomography becomes important. It is worth noting this is proposed as a follow-up strategy to define which patients should continue with or discontinue therapy [26, 27].

**Table 4** lists the recommendations by consensus for the management of arrhythmias related to cardiac sarcoidosis [4, 27].


#### **Table 4.**

*Management of conduction malfunctions.*
