Unusual presentation of more common disease/injury
CASE REPORT
Recurrent cardiac tamponade in a young woman Shobha Manish Itolikar, Santosh Salagre, Sanat Phatak, Manish Itolikar Department of Medicine, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India Correspondence to Dr Shobha Manish Itolikar, [email protected]
SUMMARY A young woman presented with recurrent pericardial effusion, she had previously been treated with antitubercular medications. She had clinical features of systemic sclerosis (SSc) which was subsequently confirmed on further workup. She was also found to be profoundly hypothyroid. Cardiac tamponade is uncommon in both SSc as well as hypothyroidism, unlike in our patient who was found to have both of these disorders. In her case, the pericardial involvement probably ante-dated the other features of SSc.
BACKGROUND Pericardial effusion occurs in disorders such as hypothyroidism, autoimmune diseases, malignancies, infections and uraemia. Although pericardial effusion has been described in association with hypothyroidism, it is rarely known to cause tamponade. Likewise, in systemic sclerosis (SSc), cardiac tamponade is not a common occurrence.
CASE PRESENTATION A 35-year-old woman presented with gradually progressive breathlessness since the past 3 months, it was accompanied by a dry cough and swelling of the feet. Her symptoms had worsened over the past 4 days and she presented with symptoms of orthopnoea. She had similar symptoms twice in the past, the first episode was 2 years ago, when she was diagnosed to have massive pericardial effusion. Since she was in tamponade, the pericardial fluid
was drained under ultrasound guidance and she was prescribed a course of antitubercular treatment. The earlier episode was 5 months ago for which she had undergone an emergency pericardiocentesis at a local hospital. There was no history of fever, chest pain, palpitations or syncope. On examination, she had tachycardia and her blood pressure was 100/70 mm Hg with an exaggerated inspiratory decline suggestive of pulsus paradoxus. Her jugular venous pressure was raised, heart sounds were muffled and chest auscultation revealed bilateral inspiratory crepitations. Chest radiograph showed cardiomegaly with well-defined heart borders (figure 1) and ECG showed low voltage complexes in all leads. We ordered a transthoracic two-dimensional (2D) echocardiography which confirmed our suspicion of massive pericardial effusion. There was fluid accumulation around the heart, causing tamponade (figure 2). There was no inspiratory collapse of the inferior vena cava noted. She underwent an emergency pericardiocentesis after which, her symptoms were relieved to a great extent. There was no echocardiographic feature suggestive of myocarditis. Her detailed examination revealed generalised skin thickening, restricted mouth opening (figure 3) and digital ulcerations (figure 4). On inquiry, she also reported symptoms of Raynaud’s phenomenon since the past year. On detailed neurological examination her higher mental functions were normal, she had no sensory or motor deficits and the tendoachilles reflex showed delayed relaxation. Skin involvement was quantified using the modified Rodnan skin score. In the 17 site score, she scored 26 of a possible maximum of 51.
INVESTIGATIONS
To cite: Itolikar SM, Salagre S, Phatak S, et al. BMJ Case Rep Published online: [ please include Day Month Year] doi:10.1136/ bcr-2013-201472
Figure 1 Cardiomegaly with well-defined heart borders on chest X-ray.
Itolikar SM, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201472
Haemogram revealed mild microcytic anaemia. Liver functions and renal functions were normal. Erythrocyte sedimentation rate was raised at 68 mm at the end of 1 h. Urinalysis was normal and did not show the presence of casts or proteinuria. Thyroid function tests revealed thyroid stimulating hormone levels of >150 IU/mL and undetectable levels of T3. Pericardial fluid was haemorrhagic in appearance with a cell count of 9600 polymorphs, 4800 lymphocytes, 128 large mononuclear cells and 1582 red blood cells. Protein content was 4.5 g/dL, sugar content 124 mg/dL and no atypical cells were seen. Her antinuclear antibody (ANA) was positive with a titre of 1:320 with a homogenous pattern on immunofluorescence. Antibodies against doublestranded DNA and U1 ribonucleoprotein were negative. Antibodies against Scleroderma-70 were positive. Baseline pulmonary function tests revealed 1
Unusual presentation of more common disease/injury
Figure 2 Two-dimensional echocardiography showing fluid surrounding the heart producing tamponade.
shrunken lung volumes and forced expiratory volume in 1 s/ forced vital capacity ratio of 82%. Pulse oxymeter measured oxygen saturations dropped from 94% to 89% on a 6 min
walking test, which was confirmed on arterial blood gas analysis. High-resolution CT scan of the chest showed bilateral patchy ground-glass opacities with pleural thickening, suggestive of interstitial lung disease (figure 5). Nail-fold capillaroscopy showed thin, enlarged, tortuous capillaries with haemorrhages (figure 6). Antimicrosomal antibodies were positive with titres of >1:1300. Antithyroid peroxidase antibody antibodies were positive. Repeat 2D echocardiography after pericardiocentesis revealed the presence of pulmonary arterial hypertension (PASP=70 mm Hg) with normal cardiac chambers. There was evidence of grade 1 diastolic dysfunction.
DIFFERENTIAL DIAGNOSIS
Figure 3 Photograph of patient’s face showing microstomia. 2
In a patient presenting with recurrent pericardial effusion, our first differential was tuberculous pericarditis, given the geographic endemicity of the disease. However, failure to respond to previously initiated empirical antitubercular therapy prompted our search for other causes. Pericardial cytology and the imaging of the chest ruled out malignancy as the cause. The patient was far too stable to explain pyogenic pericarditis. Both bacterial culture as well as culture for mycobacteria were obtained along with PCR for Mycobacterium tuberculosis: all these investigations turned out to be negative. History and Itolikar SM, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201472
Unusual presentation of more common disease/injury
Figure 6
Nail fold capillaroscopy showing dilated capillary loops.
TREATMENT
Figure 4 Photograph of the patient’s hand showing digital ulcerations.
routine investigations ruled out our other differentials of drug-induced pericarditis, uraemic pericarditis and postmyocardial infarction effusion.
After the initial pericardiocentesis, an intrapericardial catheter was kept in situ with daily aspiration of pericardial collection. Aspiration volume gradually decreased over the next 5 days and the catheter was removed on the sixth day, after ruling out residual pericardial effusion on 2D echocardiography. Thyroid supplementation was given at a dose of 100 μg/day. The patient had previously been given oral steroid along with the empirical antitubercular therapy—prednisolone had already been tapered to 5 mg daily and the same dose was continued, while she was initiated on cyclophosphamide pulse for the interstitial lung disease. Her symptoms of Raynaud’s improved after addition of oral Nifedipine. Low-dose ACE-inhibitor was initiated.
OUTCOME AND FOLLOW-UP The patient was discharged on the above medications and asked to follow-up monthly for cyclophosphamide pulse therapy.
DISCUSSION
Figure 5 High-resolution CT scan of the chest showing bilateral patchy ground-glass opacities with pleural thickening, suggestive of interstitial lung disease. Itolikar SM, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201472
SSc is a multisystemic disease characterised by skin induration and thickening, accompanied by various degrees of tissue fibrosis and chronic inflammatory infiltration in numerous visceral organs, prominent fibroproliferative vasculopathy and humoral and cellular immune alterations. Virtually every organ is affected in SSc. Although it is most obvious in the skin, the gastrointestinal tract, respiratory, renal, cardiovascular and genitourinary systems; numerous vascular structures also bear the brunt of the disease process. Based on the pattern of skin involvement as well as clinical and laboratory manifestations, patients can be classified into two principal subsets, that is, diffuse cutaneous systemic sclerosis (dcSSc) and limited cutaneous systemic sclerosis (lcSSc). While dcSSc is associated with prominent and early internal organ involvement, lcSSc presents with long-standing Raynaud’s phenomenon, indolent skin changes, limited internal organ involvement and a better prognosis.1 The distinguishing pathological hallmark of SSc is the combination of widespread capillary loss and obliterative vasculopathy of small arteries and arterioles, together with fibrosis in the skin and internal organs especially the heart, lungs, kidneys and intestinal tract. Fibrosis also occurs in the tendon sheath, perifascicular tissue surrounding skeletal muscle and some endocrine organs like the thyroid gland. Cardiovascular disease in SSc may be due to either primary involvement of the heart by sclerosing disease or secondary to involvement from disease of the kidney or lung. The 3
Unusual presentation of more common disease/injury cardiovascular involvement may be seen in the form of myocardial fibrosis, necrosis (ischaemia), conduction abnormalities, systolic and diastolic dysfunction, pericarditis and pericardial effusion (tamponade).2 Scleroderma pericardial disease is usually silent and benign. The incidence of pericardial involvement in scleroderma is about 50% according to autopsy results, but symptomatic pericarditis manifests in about 16% of patients with dcSSc and in about 30% of patients with lcSSc. Clinically evident pericardial effusion is rare in scleroderma, although it can be detected in about 41% of patients with echocardiography.3 In a majority of the patients, the pericardial effusion is small, asymptomatic and occurs after the manifestation of the other clinical and serological features of scleroderma. Although pericardial disease has been majorly described in association with renal failure in as many as 2/3rd of patients, some patients may develop fibrofibrinous pericarditis for which no other apparent cause is evident. Pericardial effusion presenting with tamponade could be attributable to the presence of severe pulmonary arterial hypertension with or without underlying lung disease.4 In this case the mortality may be as high as 55%. The fluid characteristics in either case has been found to be similar to that of plasma, both cytochemically and electrophoretically. Cardiac tamponade may rarely precede the skin manifestations of SSc.3 The pericarditis of dcSSc especially in cases with antitopoisomerase-1 may present as a chronic form with poor prognosis. Antibody negative cases may have a protracted course and may not significantly contribute to disability over long periods of time although rarely cardiac constriction may result from recurrent pericarditis.5 Our patient had recurrent pericardial effusion with tamponade well before the onset of other features of SSc. The thyroid abnormality noted in her case could be attributed to either fibrosis or autoimmune destruction of the thyroid gland by the disease process.1 The severe hypothyroidism could also have contributed to the development of pericardial effusion in
addition to the high pulmonary arterial pressures, since she had no evidence of scleroderma renal crisis.
Learning points ▸ In female participants with recurrent pericardial effusion, autoimmune diseases should be suspected. ▸ Pericardial involvement in systemic sclerosis is often subclinical. ▸ Cardiac tamponade can be a rare presentation of systemic sclerosis, sometimes even antedating the other classical features. ▸ Pericardial effusion in systemic sclerosis is largely seen in the setting of Scleroderma Renal Crisis but pulmonary hypertension ( primary or secondary) should also be sought as a causative factor.
Contributors SMI and SP were involved in collecting data and writing the case report. SMI, SS, SP and MI were involved in indoor management of the patient. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2 3 4 5
Longo DL, Fauci AS, Kasper DI, et al. Harrison’s principles of internal medicine. 18th edn. New York: McGraw-Hill Medical, 2011:chapter 323. Kahan A, Coghlan G, McLaughlin V. Cardiac complications of systemic sclerosis. Rheumatology 2009;48(Suppl 3):iii45–8. Gowda RM, Khan IA, Sacchi TJ, et al. Scleroderma pericardial disease presented with a large pericardial effusion—a case report. Angiology 2001;52:59–62. Dunne JV, Chou JP, Vishwanathan M, et al. Cardiac tamponade and large pericardial effusion in systemic sclerosis. Clin Rheumatol 2011;30:433–8. Meltzer JI. Pericardial effusion in generalised scleroderma. Am J Med 1956;20:638–42.
Copyright 2013 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
4
Itolikar SM, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201472
CASE REPORT
Recurrent cardiac tamponade in a young woman Shobha Manish Itolikar, Santosh Salagre, Sanat Phatak, Manish Itolikar Department of Medicine, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India Correspondence to Dr Shobha Manish Itolikar, [email protected]
SUMMARY A young woman presented with recurrent pericardial effusion, she had previously been treated with antitubercular medications. She had clinical features of systemic sclerosis (SSc) which was subsequently confirmed on further workup. She was also found to be profoundly hypothyroid. Cardiac tamponade is uncommon in both SSc as well as hypothyroidism, unlike in our patient who was found to have both of these disorders. In her case, the pericardial involvement probably ante-dated the other features of SSc.
BACKGROUND Pericardial effusion occurs in disorders such as hypothyroidism, autoimmune diseases, malignancies, infections and uraemia. Although pericardial effusion has been described in association with hypothyroidism, it is rarely known to cause tamponade. Likewise, in systemic sclerosis (SSc), cardiac tamponade is not a common occurrence.
CASE PRESENTATION A 35-year-old woman presented with gradually progressive breathlessness since the past 3 months, it was accompanied by a dry cough and swelling of the feet. Her symptoms had worsened over the past 4 days and she presented with symptoms of orthopnoea. She had similar symptoms twice in the past, the first episode was 2 years ago, when she was diagnosed to have massive pericardial effusion. Since she was in tamponade, the pericardial fluid
was drained under ultrasound guidance and she was prescribed a course of antitubercular treatment. The earlier episode was 5 months ago for which she had undergone an emergency pericardiocentesis at a local hospital. There was no history of fever, chest pain, palpitations or syncope. On examination, she had tachycardia and her blood pressure was 100/70 mm Hg with an exaggerated inspiratory decline suggestive of pulsus paradoxus. Her jugular venous pressure was raised, heart sounds were muffled and chest auscultation revealed bilateral inspiratory crepitations. Chest radiograph showed cardiomegaly with well-defined heart borders (figure 1) and ECG showed low voltage complexes in all leads. We ordered a transthoracic two-dimensional (2D) echocardiography which confirmed our suspicion of massive pericardial effusion. There was fluid accumulation around the heart, causing tamponade (figure 2). There was no inspiratory collapse of the inferior vena cava noted. She underwent an emergency pericardiocentesis after which, her symptoms were relieved to a great extent. There was no echocardiographic feature suggestive of myocarditis. Her detailed examination revealed generalised skin thickening, restricted mouth opening (figure 3) and digital ulcerations (figure 4). On inquiry, she also reported symptoms of Raynaud’s phenomenon since the past year. On detailed neurological examination her higher mental functions were normal, she had no sensory or motor deficits and the tendoachilles reflex showed delayed relaxation. Skin involvement was quantified using the modified Rodnan skin score. In the 17 site score, she scored 26 of a possible maximum of 51.
INVESTIGATIONS
To cite: Itolikar SM, Salagre S, Phatak S, et al. BMJ Case Rep Published online: [ please include Day Month Year] doi:10.1136/ bcr-2013-201472
Figure 1 Cardiomegaly with well-defined heart borders on chest X-ray.
Itolikar SM, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201472
Haemogram revealed mild microcytic anaemia. Liver functions and renal functions were normal. Erythrocyte sedimentation rate was raised at 68 mm at the end of 1 h. Urinalysis was normal and did not show the presence of casts or proteinuria. Thyroid function tests revealed thyroid stimulating hormone levels of >150 IU/mL and undetectable levels of T3. Pericardial fluid was haemorrhagic in appearance with a cell count of 9600 polymorphs, 4800 lymphocytes, 128 large mononuclear cells and 1582 red blood cells. Protein content was 4.5 g/dL, sugar content 124 mg/dL and no atypical cells were seen. Her antinuclear antibody (ANA) was positive with a titre of 1:320 with a homogenous pattern on immunofluorescence. Antibodies against doublestranded DNA and U1 ribonucleoprotein were negative. Antibodies against Scleroderma-70 were positive. Baseline pulmonary function tests revealed 1
Unusual presentation of more common disease/injury
Figure 2 Two-dimensional echocardiography showing fluid surrounding the heart producing tamponade.
shrunken lung volumes and forced expiratory volume in 1 s/ forced vital capacity ratio of 82%. Pulse oxymeter measured oxygen saturations dropped from 94% to 89% on a 6 min
walking test, which was confirmed on arterial blood gas analysis. High-resolution CT scan of the chest showed bilateral patchy ground-glass opacities with pleural thickening, suggestive of interstitial lung disease (figure 5). Nail-fold capillaroscopy showed thin, enlarged, tortuous capillaries with haemorrhages (figure 6). Antimicrosomal antibodies were positive with titres of >1:1300. Antithyroid peroxidase antibody antibodies were positive. Repeat 2D echocardiography after pericardiocentesis revealed the presence of pulmonary arterial hypertension (PASP=70 mm Hg) with normal cardiac chambers. There was evidence of grade 1 diastolic dysfunction.
DIFFERENTIAL DIAGNOSIS
Figure 3 Photograph of patient’s face showing microstomia. 2
In a patient presenting with recurrent pericardial effusion, our first differential was tuberculous pericarditis, given the geographic endemicity of the disease. However, failure to respond to previously initiated empirical antitubercular therapy prompted our search for other causes. Pericardial cytology and the imaging of the chest ruled out malignancy as the cause. The patient was far too stable to explain pyogenic pericarditis. Both bacterial culture as well as culture for mycobacteria were obtained along with PCR for Mycobacterium tuberculosis: all these investigations turned out to be negative. History and Itolikar SM, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201472
Unusual presentation of more common disease/injury
Figure 6
Nail fold capillaroscopy showing dilated capillary loops.
TREATMENT
Figure 4 Photograph of the patient’s hand showing digital ulcerations.
routine investigations ruled out our other differentials of drug-induced pericarditis, uraemic pericarditis and postmyocardial infarction effusion.
After the initial pericardiocentesis, an intrapericardial catheter was kept in situ with daily aspiration of pericardial collection. Aspiration volume gradually decreased over the next 5 days and the catheter was removed on the sixth day, after ruling out residual pericardial effusion on 2D echocardiography. Thyroid supplementation was given at a dose of 100 μg/day. The patient had previously been given oral steroid along with the empirical antitubercular therapy—prednisolone had already been tapered to 5 mg daily and the same dose was continued, while she was initiated on cyclophosphamide pulse for the interstitial lung disease. Her symptoms of Raynaud’s improved after addition of oral Nifedipine. Low-dose ACE-inhibitor was initiated.
OUTCOME AND FOLLOW-UP The patient was discharged on the above medications and asked to follow-up monthly for cyclophosphamide pulse therapy.
DISCUSSION
Figure 5 High-resolution CT scan of the chest showing bilateral patchy ground-glass opacities with pleural thickening, suggestive of interstitial lung disease. Itolikar SM, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201472
SSc is a multisystemic disease characterised by skin induration and thickening, accompanied by various degrees of tissue fibrosis and chronic inflammatory infiltration in numerous visceral organs, prominent fibroproliferative vasculopathy and humoral and cellular immune alterations. Virtually every organ is affected in SSc. Although it is most obvious in the skin, the gastrointestinal tract, respiratory, renal, cardiovascular and genitourinary systems; numerous vascular structures also bear the brunt of the disease process. Based on the pattern of skin involvement as well as clinical and laboratory manifestations, patients can be classified into two principal subsets, that is, diffuse cutaneous systemic sclerosis (dcSSc) and limited cutaneous systemic sclerosis (lcSSc). While dcSSc is associated with prominent and early internal organ involvement, lcSSc presents with long-standing Raynaud’s phenomenon, indolent skin changes, limited internal organ involvement and a better prognosis.1 The distinguishing pathological hallmark of SSc is the combination of widespread capillary loss and obliterative vasculopathy of small arteries and arterioles, together with fibrosis in the skin and internal organs especially the heart, lungs, kidneys and intestinal tract. Fibrosis also occurs in the tendon sheath, perifascicular tissue surrounding skeletal muscle and some endocrine organs like the thyroid gland. Cardiovascular disease in SSc may be due to either primary involvement of the heart by sclerosing disease or secondary to involvement from disease of the kidney or lung. The 3
Unusual presentation of more common disease/injury cardiovascular involvement may be seen in the form of myocardial fibrosis, necrosis (ischaemia), conduction abnormalities, systolic and diastolic dysfunction, pericarditis and pericardial effusion (tamponade).2 Scleroderma pericardial disease is usually silent and benign. The incidence of pericardial involvement in scleroderma is about 50% according to autopsy results, but symptomatic pericarditis manifests in about 16% of patients with dcSSc and in about 30% of patients with lcSSc. Clinically evident pericardial effusion is rare in scleroderma, although it can be detected in about 41% of patients with echocardiography.3 In a majority of the patients, the pericardial effusion is small, asymptomatic and occurs after the manifestation of the other clinical and serological features of scleroderma. Although pericardial disease has been majorly described in association with renal failure in as many as 2/3rd of patients, some patients may develop fibrofibrinous pericarditis for which no other apparent cause is evident. Pericardial effusion presenting with tamponade could be attributable to the presence of severe pulmonary arterial hypertension with or without underlying lung disease.4 In this case the mortality may be as high as 55%. The fluid characteristics in either case has been found to be similar to that of plasma, both cytochemically and electrophoretically. Cardiac tamponade may rarely precede the skin manifestations of SSc.3 The pericarditis of dcSSc especially in cases with antitopoisomerase-1 may present as a chronic form with poor prognosis. Antibody negative cases may have a protracted course and may not significantly contribute to disability over long periods of time although rarely cardiac constriction may result from recurrent pericarditis.5 Our patient had recurrent pericardial effusion with tamponade well before the onset of other features of SSc. The thyroid abnormality noted in her case could be attributed to either fibrosis or autoimmune destruction of the thyroid gland by the disease process.1 The severe hypothyroidism could also have contributed to the development of pericardial effusion in
addition to the high pulmonary arterial pressures, since she had no evidence of scleroderma renal crisis.
Learning points ▸ In female participants with recurrent pericardial effusion, autoimmune diseases should be suspected. ▸ Pericardial involvement in systemic sclerosis is often subclinical. ▸ Cardiac tamponade can be a rare presentation of systemic sclerosis, sometimes even antedating the other classical features. ▸ Pericardial effusion in systemic sclerosis is largely seen in the setting of Scleroderma Renal Crisis but pulmonary hypertension ( primary or secondary) should also be sought as a causative factor.
Contributors SMI and SP were involved in collecting data and writing the case report. SMI, SS, SP and MI were involved in indoor management of the patient. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2 3 4 5
Longo DL, Fauci AS, Kasper DI, et al. Harrison’s principles of internal medicine. 18th edn. New York: McGraw-Hill Medical, 2011:chapter 323. Kahan A, Coghlan G, McLaughlin V. Cardiac complications of systemic sclerosis. Rheumatology 2009;48(Suppl 3):iii45–8. Gowda RM, Khan IA, Sacchi TJ, et al. Scleroderma pericardial disease presented with a large pericardial effusion—a case report. Angiology 2001;52:59–62. Dunne JV, Chou JP, Vishwanathan M, et al. Cardiac tamponade and large pericardial effusion in systemic sclerosis. Clin Rheumatol 2011;30:433–8. Meltzer JI. Pericardial effusion in generalised scleroderma. Am J Med 1956;20:638–42.
Copyright 2013 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
4
Itolikar SM, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-201472
