| Abstract|| |
Sarcoidosis is a systemic disease of young adults. Cardiac involvement is rarely diagnosed clinically. In most cases it presents with arrhythmias and conduction disorders. We report a case of sudden death of a young female, wherein sarcoidosis with prominent cardiac involvement was diagnosed at autopsy. The other organs involved were lung and liver. Cardiac sarcoidosis should be considered in young patients with unexplained conduction disorders.
Keywords: Autopsy, cardiac sarcoidosis, myocarditis, sudden death
|How to cite this article:|
Suranagi VV, Malur PR, Bannur HB. Cardiac sarcoidosis causing sudden death. Indian J Pathol Microbiol 2009;52:566-7
| Introduction|| |
Granulomatous myocarditis is a term reserved for an entity in with well defined granulomas and should be differentiated from giant cell myocarditis (GCM). In cases of granuloma formation and multiorgan involvement, sarcoidosis and tuberculosis should be considered; the former especially when granulomas are not associated with necrosis.
We report a sudden death case of a female with granulomatous myocarditis and propose that cardiac sarcoidosis could have been the underlying etiology. Differential diagnosis for granulomatous myocarditis including sarcoidosis, tuberculosis as well as idiopathic giant cell myocarditis is discussed.
| Case Report|| |
A 39-year-old female suddenly collapsed at home. She was brought dead to the hospital. Enquiry revealed no significant symptoms or ill health before her death.
Gross lesions in the heart: Heart weighed 300gms and showed small grey white lesions measuring between 0.3-0.5cm in diameter, beneath the epicardium affecting left ventricular wall, inter-ventricular septum and atrial wall [Figure 1]. The cardiac chambers, valves, coronaries and pericardium were normal. Light microscopic examination revealed granulomatous lesions composed of lymphocytes and epithelioid cells in the myocardium, accompanied by fibrosis without any obvious necrosis [Figure 2]a. Many multinucleated giant cells of Langhan's and foreign body type were scattered in these lesions. Striations could not be seen within these giant cells. Serial sections through inter-ventricular septum revealed extensive granulomatous infiltration surrounding the conduction system.
Following this, extensive search was made for the evidence of similar lesions in other organs which revealed tiny lesions on liver and lower lobe of left lung. Histologically, these lesions showed granulomatous reaction with aggregation of giant cells and epithelioid cells, without accompanying necrosis [Figure 2]b and c. However, hilar lymph nodes and other organs did not reveal such lesions. Attempts to demonstrate acid fast bacilli to rule out tuberculosis by Ziehl Neelsen stain yielded negative results. Unfortunately, since acid fast bacilli (AFB) positivity is an infrequent finding in tuberculosis in tissue sections, polymerase chain reaction (PCR) of tissues for M. tuberculosis was performed which was negative. A diagnosis of cardiac sarcoidosis was proposed, considering the non necrotizing granulomas and extra cardiac involvement.
| Discussion|| |
Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. It commonly involves lymph nodes, lungs, eyes and skin.  Majority of the cases occur in young adults. ,, Our case was a young patient with involvement of heart, lung and liver. Similar findings were seen in other autopsy studies also.  Cardiac involvement is rarely diagnosed clinically. It presents with arrhythmias, conduction disorders, increasing myocardial insufficiency or sudden death.  More than 30% of the patients with sarcoidosis have extra pulmonary presentation of the disease.
Cardiac sarcoidosis may be isolated or associated with systemic involvement. Though lung and lymph node are common sites affected, cardiac involvement is found in 20-50% of autopsied cases with sarcoidosis. However, it only gives rise to clinical manifestations in five per cent of patients.  Cardiac sarcoidosis induces heart failure or sudden death in many cases and is thus often associated with poor prognosis. Sudden death is the initial presentation in approximately 20% of the patients.  In the absence of extra cardiac signs, it is difficult to diagnose clinically. Cardiac sarcoidosis should be considered in young patients with unexplained conduction disorders. 
The main differential diagnoses in a case of sudden death in a young patient with granulomatous myocarditis would be tuberculosis, sarcoidosis and giant cell myocarditis. Idiopathic GCM which used to be classified with granulomatous lesion before is now thought to be a separate entity from granulomatous myocarditis since, it lacks true granuloma formation. Typical multifocal or wide spread geographic necrosis, presence of myogenic giant cells, prominent eosinophilic infiltration and absence of extra cardiac granulomas or giant cells observed in GCM  differentiate this condition from sarcoidosis. Our case lacked the above features making GCM an unlikely diagnosis. The concept that GCM and cardiac sarcoidosis are two different entities has been challenged due to inadequate appraisal of extra cardiac organ system.  However, immunohistochemical evidence suggest that giant cells of GCM are myogenic in origin, being derived from altered myocytes rather than from tissue macrophages.  The characteristic giant cells of GCM are uniformly negative for cytoplasmic muramidase (CM) and positive for myoglobin whereas CM positive in tissue macrophages and giant cells of generalized sarcoidosis invading the heart.  These findings suggest and support the concept of classifying GCM as a separate entity from generalized sarcoidosis.
Tuberculosis, initially considered a close differential diagnosis of extra cardiac granulomatous lesions involving lung and liver was also unlikely in this case because there was very minimal to absent necrosis. Identification of mycobacterium by conventional stains, culture or both is necessary to confirm the diagnosis. Culture was not done in this case since fixed organs were received for autopsy examination. However, acid fast bacilli were not present and PCR, for M. tuberculosis was negative, further supporting the exclusion of this diagnosis in this case. ,
The characteristic microscopic features of sarcoidosis are well formed, non necrotizing granulomatous lesions with giant cells, dense fibrosis, extracardiac lesions being commonly seen. In chronic cases, repeated dissemination, particularly interstitial spread of granulomatous changes, leads to prominent interstitial fibrosis and dysfunction of organs resulting in death of individual.
| Acknowledgment|| |
The authors wish to express their thanks to Dr. Jaya Deshpande, consultant pathologist, Metropolis, Mumbai, for her valuable opinion in the diagnosis of the case.
We would also wish to thank Dr. Hemshettar, microbiologist, Hitech Laboratory, Belgaum for help rendered during PCR studies required for this case.
| References|| |
|1.||Veinot JP, Johnston B. Cardiac sarcoidosis: An occult cause of sudden death: A case report and literature review. J Forensic Sci 1998;43:715-7. |
|2.||Kollerman J, Roos G, Helpap B. Sudden cardiac death from unrecognized cardiac sarcoidosis. Pathologe 2001;22:141-4. |
|3.||Wan Muhaizan WM, Swaminathan M, Daud MS. Cardiac sarcoidosis: Two cases with autopsy findings. Malays J Pathol 2004;26:59-63. |
|4.||Cooper LT, Berry GJ, Shabetai R. Idiopathic Giant cell myocarditis: Natural history and treatment. N Engl J Med 1997;336:1860-6. |
|5.||Tubbs RR, Sheibani K, Hawk WA. Giant cell myocarditis. Arch Pathol Lab Med 1980;104:245-6. |
|6.||Tanaka M, Ichinohasama R, Kawahara Y, Esaki Y, Hirokawa K, Okishige K, et al. Acute idiopathic interstitial myocarditis: Case report with special reference to morphological characteristics of giant cells. Clin Pathol 1986;39:1209-16. |
|7.||Liz, Qin W, Yue Q, Meng K, Lin Q. Study on detection of Mycobacterium DNA in formalin fixed paraffin embedded tissue samples by triplex polymerase chain reaction. Wei Sheng Wu Xue Bao 2002;42:69-75. |
|8.||Hsiao PF, Tzen CY, Chen HC, Su HY. Polymerase chain reaction based detection of M. Tuberculosis in tissues showing granulomatous inflammation without demonstrable acid fast bacilli. Int J Dermatol 2003;42:281-6. |
Vijayalaxmi V Suranagi
A-14/11, JNMC Staff Quarters, Nehru nagar, Belgaum-590 010, Karnataka
[Figure 1], [Figure 2]