Rheumatol Int DOI 10.1007/s00296-014-2958-6
Short Communication
Pericardial effusion and giant cell arteritis Thierry Zenone · Marie Puget
Received: 12 December 2013 / Accepted: 29 January 2014 © Springer-Verlag Berlin Heidelberg 2014
Abstract Pericardial effusion in patients with giant cell arteritis has been described in case reports. The exact prevalence of this manifestation is unknown. The objective of this study was to determine the prevalence and characteristics of pericardial effusion in patients with giant cell arteritis. Retrospective data were collected from 114 patients at a single hospital with giant cell arteritis diagnosed between October 1999 and July 2013. Pericardial effusion was found in four patients (3.5 %, 95 % confidence intervals 1–8.7). In three cases, pericardial effusion was present at initial presentation and was asymptomatic. In one case, pericardial effusion was associated with aortitis and diagnosed at 6 months on thoracic CT scan because of relapse of the disease when corticosteroid was reduced. Prognosis was good in all cases. The present study confirms that pericardial effusion could occur in patients with giant cell arteritis. However, pericardial effusion is a rare manifestation of giant cell arteritis. Clinical course of pericardial effusion in the setting of giant cell arteritis is usually benign and rarely symptomatic. Recognition of this manifestation may contribute to early diagnosis of the disease. Keywords Giant cell arteritis · Pericarditis · Vasculitis · Corticosteroid
Introduction Giant cell arteritis (GCA) is a chronic vasculitis of largeand medium-sized vessels predominantly involving the
T. Zenone (*) · M. Puget Unit of Internal Medicine, Department of Medicine, Centre Hospitalier General, 179 boulevard Marechal Juin, 26953 Valence Cedex 9, France e-mail: tzenone@ch‑valence.fr
branches of the aortic wall. It is the most common form of systemic vasculitis in adults over the age of 50 years. The typical clinical manifestations of GCA are new headache, jaw claudication, fever, weight loss, symptoms of polymyalgia rheumatica (PMR) and visual loss. Diagnosis of GCA is based on clinical features and elevated levels of inflammatory biomarkers. Temporal artery biopsy (TAB) remains the gold standard to support the diagnosis of GCA; imaging studies are useful to delineate large-vessel involvement. Corticosteroids remain the cornerstone of treatment of GCA [1]. GCA rarely causes cardiac problems, but the increased related risk to develop thoracic aortic aneurysm and aortic dissection is now well known. The frequency of these findings is rather low at the time of diagnosis, yet considerably higher during follow-up [2]. GCA can also cause coronary arteritis and myocardial infarction, but it is not known how often this occurs [2]. Although pericardial effusion at the time of diagnosis in patients with GCA has been described in several case reports [3–20], information on this issue on large series of individuals is more limited and the exact prevalence of this manifestation in GCA is unknown. Pericardial effusion seems to be an extremely rare manifestation of GCA [15]. Considering these issues, we conducted a single centre retrospective chart review of all patients evaluated at our department of internal medicine for GCA (biopsy-proven or not, but defined by American College of Rheumatology (ACR) classification criteria [21]) between October 1999 and July 2013. The objective of this study was to determine the prevalence and the characteristics of pericardial effusion in patients with GCA. The relevant literature concerning this particular manifestation is also briefly reviewed.
13
Patients and methods Definitions and study design From October 1999 to July 2013, we gathered a series of consecutive patients with GCA (only incident cases), who had presented to the unit of internal medicine at our hospital. Only patients who fulfilled the classification criteria as formulated by the ACR [21] were included in the study (inclusion criteria). All data were collected from medical charts and recorded on a standardized data collection form.
Rheumatol Int
claudication, PMR, visual loss, pleural and pericardial effusion, thoracic aortic aneurysm, aortitis and stroke) and biological data (ESR, CRP) for all the patients with GCA. Data analysis We used descriptive statistics to characterize our population in this study. Quantitative variables were described as means, standard deviation (SD) and medians, and categorical variables as percentage with 95 % confidence intervals (CIs).
Evaluation and data collection Results All patients with suspected GCA seen at our unit of internal medicine were evaluated by two senior practitioners (the authors) and underwent the following evaluations: • history taking, thorough physical examination • erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complete blood cell count, renal function, serum levels of electrolytes, glucose, total protein, albumin, globulin, liver function • chest X-ray, electrocardiogram (EKG) • thoracic and abdominal computed tomographic (CT) scan • unilateral TAB. The side with predominant symptoms and signs was selected for biopsy • in case of visual disturbance, complete ophthalmologic examination • if indicated, in order to demonstrate large-vessel inflammation such as aortitis, 18F-fluorodeoxyglucose positron emission tomography (FDG-PET). Two-dimensional echocardiography is usually not included in the work-up of GCA. However, two-dimensional echocardiography was performed in 83 patients in our survey mainly in the initial work-up of patients with fever of unknown origin in order to rule out infective endocarditis [22]. So, pericardial effusion was defined using chest X-ray (performed in all cases), EKG (performed in all cases), echocardiography (performed in 83 cases 73 %) and thoracic CT scan (performed in 104 patients 91 %). Ten patients had only EKG and chest X-ray; 104 patients had either echocardiography or thoracic CT scan. Investigations (EKG, chest X-ray, echocardiography and thoracic CT scan) were done before treatment with glucocorticoids in all cases. FDG-PET was performed in 19 patients. We analysed the demographic data (sex and age at diagnosis), clinical data (constitutional symptoms, fever (>38 °C), headache, abnormal temporal artery on physical examination, cervical pain, peripheral arthritis, jaw
13
Description of the cohort Between October 1999 and July 2013, 114 patients with GCA matched all inclusion criteria and were retained for analysis. Of the 114 patients, 75 (65.8 %) were females and 39 (34.2 %) were males. The patient’s mean age was 75.6 years (SD 7.8). The median age was 77 years, ranging from 59 to 92. Abnormal findings on TAB in favour of the diagnosis of GCA were found in 81 patients, and in one case, diagnosis of GCA was histologically confirmed after surgery of thoracic aortic aneurysm. In 32 patients (28 %), TAB was normal; the diagnosis of GCA with “negative TAB” was made. The clinical manifestations at initial presentation of the 114 patients are presented in Table 1. The mean ESR was 79.5 mm/h (SD 27.1). The median ESR was 88.5 mm/h, ranging from 2 to 140. The mean CRP was 107 mg/l (SD 78.1). The median CRP was 80 mg/l, ranging from 1 to 361. Frequency and characteristics of pericardial effusion in patients with GCA In our series of 114 patients with GCA, pericardial effusion was found in four patients (3.5 %, 95 % CI 1–8.7). The principal data of the four patients are presented in Table 2. Inflammatory biomarkers were higher in patients with pericardial effusion (mean ESR 108 mm/h; mean CRP 144 mg/l). In three cases, pericardial effusion was present at initial presentation (2.6 %). In one case, pericardial effusion was associated with aortitis and diagnosed at 6 months on thoracic CT scan because of relapse of the disease when corticosteroid was reduced (Fig. 1a, b). Patients were asymptomatic in all cases (no chest pain and no dyspnoea). Chest X-ray and EKG were normal in all cases. Pericardial effusion was detected by chance after two-dimensional echocardiography or thoracic CT scan performed for other reasons. We cannot prove that there was no pericardial effusion in the 10 patients who only had EKG and chest X-ray.
Favourable steroid and methotrexate at 3 years
Favourable 8 months
Favourable 8 months
M/62 4
98 100 Headache, jaw claudication, neck pain
98 100 Fatigue, inflammation of unknown origin
Normal
M/80 3
Typical GCA
F/67 2
Typical GCA
109 F/64 1
The true prevalence and clinical importance of cardiac abnormalities in GCA, both at time of presentation and during evolution of the disease, is difficult to delineate from the existing literature data. Aortic and coronary artery involvement is well described in GCA, but pericardial effusion is a very unusual feature. To the best of our knowledge, this manifestation of GCA was first reported in 1972 by Miller [3], and during the last four decades, 10 % body weight
GCA giant cell arteritis, ESR erythrocyte sedimentation rate, CRP C-reactive protein, TAB temporal artery biopsy, EKG electrocardiogram, CT computed tomographic scan
a
Asymptomatic, at 8-month EKG: normal, echocardiography and thoracic CT scan: aortitis and pericardial effusion (10 mm)
2 (1.8)
234
Pleural effusion
123
3 (2.6) 29 (25.4) 49 (43) 33 (28.9) 16 (14) 9 (7.9) 7 (6.1) 3 (2.6)
Fatigue, dry cough, weight loss
Peripheral arthritis Temporal arteries abnormalities Fever Weight lossa Ischaemia of the optic nerve Aortitis Stroke Thoracic aortic aneurysm
Asymptomatic, at initial presentation EKG: normal, echocardiography and thoracic CT scan: moderate anterior pericardial effusion (8 mm) Asymptomatic, at initial presentation EKG: normal, echocardiography and thoracic CT scan: moderate anterior and apical pericardial effusion (6 mm) Asymptomatic, at initial presentation EKG: normal, echocardiography and thoracic CT scan: moderate anterior pericardial effusion (5 mm)
83 (72.8) 39 (34.2) 32 (28.1) 33 (28.9)
Normal
New headache Jaw claudication Cervical pain Polymyalgia rheumatica
146
No. (%)
Headache, fever, abnormal temporal arteries, neck pain
Characteristics
Outcome follow-up
Table 1 Clinical manifestations of giant cell arteritis in the study population
Favourable steroid withdrawal at 3 years
Rheumatol Int
13
Rheumatol Int
Fig. 1 Thoracic CT scan: pericardial effusion (a) and thickening of aortic wall (b)
be used in the interpretation of the data due to the retrospective nature of the study and due to the fact that patients were not uniformly diagnosed (echocardiography was performed in 73 % of patients and thoracic CT scan in 91 % of patients). In our cases, it seems likely that the pericardial effusion was a feature of GCA, since detailed investigation revealed no other known cause and we have evidence that the effusion resolved following corticosteroid therapy. The present study also demonstrates that pericardial effusion is usually asymptomatic with good prognosis and favourable outcome with treatment. No severe complication of pericarditis has been reported in the literature on corticosteroid therapy [17]. We show that EKG and chest X-ray did not pick up any of the four pericardial effusions that were seen in this series. Unusually, most of the patients had an echocardiography, a thoracic CT scan or both, and this is how the pericardial effusions were picked up. In case one, patient had dry cough. However, we do not believe that it was a manifestation of effusion. The mechanism of cough in GCA is unclear. Vasculitis involving the main pulmonary artery and other large- and medium-sized pulmonary vessels has been described. Some authors speculate that granulomatous involvement of the bronchial wall and/or inflammation of the peribronchial pulmonary vasculature may explain chronic cough in GCA. In a previous study, we compared 12 patients with dry cough with 76 patients without dry cough. Dry cough was not related to the presence of other thoracic symptoms (including pericardial and pleural effusions). A correlation was found between inflammatory biomarkers and presence of dry cough [23]. In the literature, in case of pericardial effusion, diagnosis is frequently delayed because of the unusual presentation without headache and with clinically normal temporal arteries [3]. None of these factors by themselves should delay the taking of a TAB and/or a FDG-PET [19].
13
Besides pericardial involvement, GCA can also atypically manifest with pleural effusion [24–26], two cases in our study. Simultaneous occurrence including both pericardial effusion and pleural effusion is extremely rare in the literature [13] with no case in our study. Pericardial effusion is sometimes associated with aortitis [11, 19, 20], as in case four. The frequency of aortitis may be underestimated in our study. Myopericarditis has been reported in two cases in the literature; patients developed global cardiac insufficiency [14]. Cases of PMR (without GCA) manifesting with pericardial effusion have been also reported in the literature [27, 28]. The pathophysiology of this manifestation is unknown. To the best of our knowledge, there has been no report confirming pathologically pericardial inflammation. The reason is the fact that there are no available biopsies in these cases. However, some hypothesis can be proposed: inflammatory cytokine storm, immune complex deposition, giant cell vasculitis of pericardial arteries or inflammatory interstitial lesion of the pericardium with or without granuloma [15, 16]. We cannot exclude the possibility that the pericardial effusion may not be related to the GCA itself but for a trigger of the GCA e.g. a prior viral infection. Although limitations related to the retrospective nature of this study may exist, the results derived from the analysis of this monocentric series of patients with GCA support the evidence that pericardial effusion may be occasionally a manifestation of GCA. Only prospective studies on systematic echocardiography when faced with the diagnosis of GCA, whatever clinical symptoms, will enable to appreciate the exact prevalence and prognosis value of this manifestation. Mild pericardial effusion may exist in healthy old individuals. So, it would be better to compare GCA patients with an age- and sex-matched healthy control group.
Rheumatol Int
Although the prevalence of pericardial effusion-related GCA is low, the diagnosis of GCA should always be considered in an elderly patient with a pericardial effusion and an unexplained elevation of inflammatory biomarkers. These patients may have few of the usual symptoms of GCA, so the diagnosis may be initially overlooked. An accurate clinical history and familiarity with typical and atypical features of GCA can help in establishing an early diagnosis, which enables prompt initiation of treatment. The prognosis of GCA is determined by how promptly the disease is diagnosed and treated. Although the clinical course of pericardial effusion in the setting of GCA is typically benign, and rarely symptomatic, recognition of this non-classical manifestation may contribute to early diagnosis of the disease. Conflict of interest None.
References 1. Salvarani C, Pipitone N, Versari A, Hunder GG (2012) Clinical features of polymyalgia rheumatica and giant cell arteritis. Nat Rev Rheumatol 8:509–521 2. Knockaert DC (2007) Cardiac involvement in systemic inflammatory diseases. Eur Heart J 28:1797–1804 3. Miller JP (1972) Pericardial effusion and giant-cell arteritis. Proc R Soc Med 65:565 4. Dupond JL, Leconte des Floris R (1982) Temporal arteritis manifested as an acute febrile pericarditis. JAMA 247:2371–2372 5. Dupasquier E (1982) Pericarditis in giant cell temporal arteritis. Rev Med Suisse Romande 102:815–822 6. Clementz GL, Gold F, Khaiser N, Zolin WD, Jalovec L (1989) Giant cell arteritis associated with pericarditis and pancreatic insufficiency in a patient with psoriatic arthritis. J Rheumatol 16:128–129 7. Guillaume M, Vachiery F, Cogan E (1991) Pericarditis: an unusual manifestation of giant cell arteritis. Am J Med 91:662–664 8. Preston J, Warner M (1991) Pericardial effusion as a manifestation of giant cell arteritis. Am J Med 91:439–440 9. Stanley D, Henderson D, Harris S (1991) Giant cell arteritis associated with pericarditis and large vessel disease. Aust N Z J Med 21:353–355 10. Pedro-Botet J, Coll J, Lopez MJ, Grau JM (1996) Pericardial effusion and giant cell arteritis. Br J Rheumatol 35:194–195
11. Pattis P, Stockner I, Amor H (2000) Pericardial effusion and aortitis: unusual main manifestations of giant cell arteritis. Acta Med Austriaca 27:83–86 12. Butler JV, Callaghan J, O’Keeffe S, Mulkerrin EC (2001) Giant cell arteritis and pericardial effusion. Ir Med J 94:87–88 13. Valstar MH, Terpstra WF, de Jong RS (2003) Pericardial and pleural effusion in giant cell arteritis. Am J Med 114:708–709 14. Teixera A, Capitaine E, Congy F, Herson S, Cherin P (2003) Myopericarditis during Horton disease. Rev Med Interne 24:189–194 15. Bablekos GD, Michaelides SA, Karachalios GN, Nicolaou IN, Batistatou AK, Charalabopoulos KA (2006) Pericardial involvement as an atypical manifestation of giant cell arteritis. Am J Med Sci 332:198–204 16. Guindon A, Rossi P, Bagneres D et al (2007) Pericarditis: a giant cell arteritis manifestation. Rev Med Interne 28:326–331 17. Moulis G, Sailler L, Astudillo L, Vernet J, Couret B, Arlet P (2010) Pericarditis as the presenting manifestation of giant cell arteritis. Rev Med Interne 31:46–48 18. Blot M, Guépet H, Aubriot-Lorton MH, Pfitzenmeyer P, Manckoundia P (2010) An atypical case of giant cell arteritis (Horton’s disease) associated with facial swelling, confusion, and pericarditis in an elderly woman. J Am Geriatr Soc 58:2040–2041 19. Couturier B, Huyge V, Soyfoo MS (2011) Pericardi tis revealing large vessel vasculitis. ISRN Rheumatol. doi:10.5402/2011/648703 20. Matsue Y, Ohno M, Nagahori W, Suzuki M, Matsumura A, Hashimoto Y (2011) A case of giant cell arteritis with massive pericardial effusion. Heart Vessels 26:562–564 21. Hunder CG, Bloch DA, Michel BA et al (1990) The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 33:1122–1128 22. Zenone T (2006) Fever of unknown origin in adults: evaluation of 144 cases in a non-university hospital. Scand J Infect Dis 38:625–631 23. Zenone T, Puget M (2013) Dry cough is a frequent manifestation of giant cell arteritis. Rheumatol Int 33:2165–2168 24. Ramos A, Laguna P, Cuervas V (1992) Pleural effusion in giant cell arteritis. Ann Intern Med 116:957 25. Garcia-Alfranca F, Solans R, Simenon C et al (1998) Pleural effusion as a form of presentation of temporal arteritis. Br J Rheumatol 37:802–803 26. Marie I, Heliot P, Muir JF et al (2004) Pleural effusion revealing giant cell arteritis. Eur J Intern Med 15:125–127 27. Brucato A, Brambilla G (2002) Polymyalgia rheumatica and pericardial tamponade. Ann Rheum Dis 81:283 28. Malone CB, McCarthy GM (2005) Polymyalgia rheumatica as an unusual cause of pleural and pericardial effusion. J Clin Rheumatol 11:59–60
13
Short Communication
Pericardial effusion and giant cell arteritis Thierry Zenone · Marie Puget
Received: 12 December 2013 / Accepted: 29 January 2014 © Springer-Verlag Berlin Heidelberg 2014
Abstract Pericardial effusion in patients with giant cell arteritis has been described in case reports. The exact prevalence of this manifestation is unknown. The objective of this study was to determine the prevalence and characteristics of pericardial effusion in patients with giant cell arteritis. Retrospective data were collected from 114 patients at a single hospital with giant cell arteritis diagnosed between October 1999 and July 2013. Pericardial effusion was found in four patients (3.5 %, 95 % confidence intervals 1–8.7). In three cases, pericardial effusion was present at initial presentation and was asymptomatic. In one case, pericardial effusion was associated with aortitis and diagnosed at 6 months on thoracic CT scan because of relapse of the disease when corticosteroid was reduced. Prognosis was good in all cases. The present study confirms that pericardial effusion could occur in patients with giant cell arteritis. However, pericardial effusion is a rare manifestation of giant cell arteritis. Clinical course of pericardial effusion in the setting of giant cell arteritis is usually benign and rarely symptomatic. Recognition of this manifestation may contribute to early diagnosis of the disease. Keywords Giant cell arteritis · Pericarditis · Vasculitis · Corticosteroid
Introduction Giant cell arteritis (GCA) is a chronic vasculitis of largeand medium-sized vessels predominantly involving the
T. Zenone (*) · M. Puget Unit of Internal Medicine, Department of Medicine, Centre Hospitalier General, 179 boulevard Marechal Juin, 26953 Valence Cedex 9, France e-mail: tzenone@ch‑valence.fr
branches of the aortic wall. It is the most common form of systemic vasculitis in adults over the age of 50 years. The typical clinical manifestations of GCA are new headache, jaw claudication, fever, weight loss, symptoms of polymyalgia rheumatica (PMR) and visual loss. Diagnosis of GCA is based on clinical features and elevated levels of inflammatory biomarkers. Temporal artery biopsy (TAB) remains the gold standard to support the diagnosis of GCA; imaging studies are useful to delineate large-vessel involvement. Corticosteroids remain the cornerstone of treatment of GCA [1]. GCA rarely causes cardiac problems, but the increased related risk to develop thoracic aortic aneurysm and aortic dissection is now well known. The frequency of these findings is rather low at the time of diagnosis, yet considerably higher during follow-up [2]. GCA can also cause coronary arteritis and myocardial infarction, but it is not known how often this occurs [2]. Although pericardial effusion at the time of diagnosis in patients with GCA has been described in several case reports [3–20], information on this issue on large series of individuals is more limited and the exact prevalence of this manifestation in GCA is unknown. Pericardial effusion seems to be an extremely rare manifestation of GCA [15]. Considering these issues, we conducted a single centre retrospective chart review of all patients evaluated at our department of internal medicine for GCA (biopsy-proven or not, but defined by American College of Rheumatology (ACR) classification criteria [21]) between October 1999 and July 2013. The objective of this study was to determine the prevalence and the characteristics of pericardial effusion in patients with GCA. The relevant literature concerning this particular manifestation is also briefly reviewed.
13
Patients and methods Definitions and study design From October 1999 to July 2013, we gathered a series of consecutive patients with GCA (only incident cases), who had presented to the unit of internal medicine at our hospital. Only patients who fulfilled the classification criteria as formulated by the ACR [21] were included in the study (inclusion criteria). All data were collected from medical charts and recorded on a standardized data collection form.
Rheumatol Int
claudication, PMR, visual loss, pleural and pericardial effusion, thoracic aortic aneurysm, aortitis and stroke) and biological data (ESR, CRP) for all the patients with GCA. Data analysis We used descriptive statistics to characterize our population in this study. Quantitative variables were described as means, standard deviation (SD) and medians, and categorical variables as percentage with 95 % confidence intervals (CIs).
Evaluation and data collection Results All patients with suspected GCA seen at our unit of internal medicine were evaluated by two senior practitioners (the authors) and underwent the following evaluations: • history taking, thorough physical examination • erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complete blood cell count, renal function, serum levels of electrolytes, glucose, total protein, albumin, globulin, liver function • chest X-ray, electrocardiogram (EKG) • thoracic and abdominal computed tomographic (CT) scan • unilateral TAB. The side with predominant symptoms and signs was selected for biopsy • in case of visual disturbance, complete ophthalmologic examination • if indicated, in order to demonstrate large-vessel inflammation such as aortitis, 18F-fluorodeoxyglucose positron emission tomography (FDG-PET). Two-dimensional echocardiography is usually not included in the work-up of GCA. However, two-dimensional echocardiography was performed in 83 patients in our survey mainly in the initial work-up of patients with fever of unknown origin in order to rule out infective endocarditis [22]. So, pericardial effusion was defined using chest X-ray (performed in all cases), EKG (performed in all cases), echocardiography (performed in 83 cases 73 %) and thoracic CT scan (performed in 104 patients 91 %). Ten patients had only EKG and chest X-ray; 104 patients had either echocardiography or thoracic CT scan. Investigations (EKG, chest X-ray, echocardiography and thoracic CT scan) were done before treatment with glucocorticoids in all cases. FDG-PET was performed in 19 patients. We analysed the demographic data (sex and age at diagnosis), clinical data (constitutional symptoms, fever (>38 °C), headache, abnormal temporal artery on physical examination, cervical pain, peripheral arthritis, jaw
13
Description of the cohort Between October 1999 and July 2013, 114 patients with GCA matched all inclusion criteria and were retained for analysis. Of the 114 patients, 75 (65.8 %) were females and 39 (34.2 %) were males. The patient’s mean age was 75.6 years (SD 7.8). The median age was 77 years, ranging from 59 to 92. Abnormal findings on TAB in favour of the diagnosis of GCA were found in 81 patients, and in one case, diagnosis of GCA was histologically confirmed after surgery of thoracic aortic aneurysm. In 32 patients (28 %), TAB was normal; the diagnosis of GCA with “negative TAB” was made. The clinical manifestations at initial presentation of the 114 patients are presented in Table 1. The mean ESR was 79.5 mm/h (SD 27.1). The median ESR was 88.5 mm/h, ranging from 2 to 140. The mean CRP was 107 mg/l (SD 78.1). The median CRP was 80 mg/l, ranging from 1 to 361. Frequency and characteristics of pericardial effusion in patients with GCA In our series of 114 patients with GCA, pericardial effusion was found in four patients (3.5 %, 95 % CI 1–8.7). The principal data of the four patients are presented in Table 2. Inflammatory biomarkers were higher in patients with pericardial effusion (mean ESR 108 mm/h; mean CRP 144 mg/l). In three cases, pericardial effusion was present at initial presentation (2.6 %). In one case, pericardial effusion was associated with aortitis and diagnosed at 6 months on thoracic CT scan because of relapse of the disease when corticosteroid was reduced (Fig. 1a, b). Patients were asymptomatic in all cases (no chest pain and no dyspnoea). Chest X-ray and EKG were normal in all cases. Pericardial effusion was detected by chance after two-dimensional echocardiography or thoracic CT scan performed for other reasons. We cannot prove that there was no pericardial effusion in the 10 patients who only had EKG and chest X-ray.
Favourable steroid and methotrexate at 3 years
Favourable 8 months
Favourable 8 months
M/62 4
98 100 Headache, jaw claudication, neck pain
98 100 Fatigue, inflammation of unknown origin
Normal
M/80 3
Typical GCA
F/67 2
Typical GCA
109 F/64 1
The true prevalence and clinical importance of cardiac abnormalities in GCA, both at time of presentation and during evolution of the disease, is difficult to delineate from the existing literature data. Aortic and coronary artery involvement is well described in GCA, but pericardial effusion is a very unusual feature. To the best of our knowledge, this manifestation of GCA was first reported in 1972 by Miller [3], and during the last four decades, 10 % body weight
GCA giant cell arteritis, ESR erythrocyte sedimentation rate, CRP C-reactive protein, TAB temporal artery biopsy, EKG electrocardiogram, CT computed tomographic scan
a
Asymptomatic, at 8-month EKG: normal, echocardiography and thoracic CT scan: aortitis and pericardial effusion (10 mm)
2 (1.8)
234
Pleural effusion
123
3 (2.6) 29 (25.4) 49 (43) 33 (28.9) 16 (14) 9 (7.9) 7 (6.1) 3 (2.6)
Fatigue, dry cough, weight loss
Peripheral arthritis Temporal arteries abnormalities Fever Weight lossa Ischaemia of the optic nerve Aortitis Stroke Thoracic aortic aneurysm
Asymptomatic, at initial presentation EKG: normal, echocardiography and thoracic CT scan: moderate anterior pericardial effusion (8 mm) Asymptomatic, at initial presentation EKG: normal, echocardiography and thoracic CT scan: moderate anterior and apical pericardial effusion (6 mm) Asymptomatic, at initial presentation EKG: normal, echocardiography and thoracic CT scan: moderate anterior pericardial effusion (5 mm)
83 (72.8) 39 (34.2) 32 (28.1) 33 (28.9)
Normal
New headache Jaw claudication Cervical pain Polymyalgia rheumatica
146
No. (%)
Headache, fever, abnormal temporal arteries, neck pain
Characteristics
Outcome follow-up
Table 1 Clinical manifestations of giant cell arteritis in the study population
Favourable steroid withdrawal at 3 years
Rheumatol Int
13
Rheumatol Int
Fig. 1 Thoracic CT scan: pericardial effusion (a) and thickening of aortic wall (b)
be used in the interpretation of the data due to the retrospective nature of the study and due to the fact that patients were not uniformly diagnosed (echocardiography was performed in 73 % of patients and thoracic CT scan in 91 % of patients). In our cases, it seems likely that the pericardial effusion was a feature of GCA, since detailed investigation revealed no other known cause and we have evidence that the effusion resolved following corticosteroid therapy. The present study also demonstrates that pericardial effusion is usually asymptomatic with good prognosis and favourable outcome with treatment. No severe complication of pericarditis has been reported in the literature on corticosteroid therapy [17]. We show that EKG and chest X-ray did not pick up any of the four pericardial effusions that were seen in this series. Unusually, most of the patients had an echocardiography, a thoracic CT scan or both, and this is how the pericardial effusions were picked up. In case one, patient had dry cough. However, we do not believe that it was a manifestation of effusion. The mechanism of cough in GCA is unclear. Vasculitis involving the main pulmonary artery and other large- and medium-sized pulmonary vessels has been described. Some authors speculate that granulomatous involvement of the bronchial wall and/or inflammation of the peribronchial pulmonary vasculature may explain chronic cough in GCA. In a previous study, we compared 12 patients with dry cough with 76 patients without dry cough. Dry cough was not related to the presence of other thoracic symptoms (including pericardial and pleural effusions). A correlation was found between inflammatory biomarkers and presence of dry cough [23]. In the literature, in case of pericardial effusion, diagnosis is frequently delayed because of the unusual presentation without headache and with clinically normal temporal arteries [3]. None of these factors by themselves should delay the taking of a TAB and/or a FDG-PET [19].
13
Besides pericardial involvement, GCA can also atypically manifest with pleural effusion [24–26], two cases in our study. Simultaneous occurrence including both pericardial effusion and pleural effusion is extremely rare in the literature [13] with no case in our study. Pericardial effusion is sometimes associated with aortitis [11, 19, 20], as in case four. The frequency of aortitis may be underestimated in our study. Myopericarditis has been reported in two cases in the literature; patients developed global cardiac insufficiency [14]. Cases of PMR (without GCA) manifesting with pericardial effusion have been also reported in the literature [27, 28]. The pathophysiology of this manifestation is unknown. To the best of our knowledge, there has been no report confirming pathologically pericardial inflammation. The reason is the fact that there are no available biopsies in these cases. However, some hypothesis can be proposed: inflammatory cytokine storm, immune complex deposition, giant cell vasculitis of pericardial arteries or inflammatory interstitial lesion of the pericardium with or without granuloma [15, 16]. We cannot exclude the possibility that the pericardial effusion may not be related to the GCA itself but for a trigger of the GCA e.g. a prior viral infection. Although limitations related to the retrospective nature of this study may exist, the results derived from the analysis of this monocentric series of patients with GCA support the evidence that pericardial effusion may be occasionally a manifestation of GCA. Only prospective studies on systematic echocardiography when faced with the diagnosis of GCA, whatever clinical symptoms, will enable to appreciate the exact prevalence and prognosis value of this manifestation. Mild pericardial effusion may exist in healthy old individuals. So, it would be better to compare GCA patients with an age- and sex-matched healthy control group.
Rheumatol Int
Although the prevalence of pericardial effusion-related GCA is low, the diagnosis of GCA should always be considered in an elderly patient with a pericardial effusion and an unexplained elevation of inflammatory biomarkers. These patients may have few of the usual symptoms of GCA, so the diagnosis may be initially overlooked. An accurate clinical history and familiarity with typical and atypical features of GCA can help in establishing an early diagnosis, which enables prompt initiation of treatment. The prognosis of GCA is determined by how promptly the disease is diagnosed and treated. Although the clinical course of pericardial effusion in the setting of GCA is typically benign, and rarely symptomatic, recognition of this non-classical manifestation may contribute to early diagnosis of the disease. Conflict of interest None.
References 1. Salvarani C, Pipitone N, Versari A, Hunder GG (2012) Clinical features of polymyalgia rheumatica and giant cell arteritis. Nat Rev Rheumatol 8:509–521 2. Knockaert DC (2007) Cardiac involvement in systemic inflammatory diseases. Eur Heart J 28:1797–1804 3. Miller JP (1972) Pericardial effusion and giant-cell arteritis. Proc R Soc Med 65:565 4. Dupond JL, Leconte des Floris R (1982) Temporal arteritis manifested as an acute febrile pericarditis. JAMA 247:2371–2372 5. Dupasquier E (1982) Pericarditis in giant cell temporal arteritis. Rev Med Suisse Romande 102:815–822 6. Clementz GL, Gold F, Khaiser N, Zolin WD, Jalovec L (1989) Giant cell arteritis associated with pericarditis and pancreatic insufficiency in a patient with psoriatic arthritis. J Rheumatol 16:128–129 7. Guillaume M, Vachiery F, Cogan E (1991) Pericarditis: an unusual manifestation of giant cell arteritis. Am J Med 91:662–664 8. Preston J, Warner M (1991) Pericardial effusion as a manifestation of giant cell arteritis. Am J Med 91:439–440 9. Stanley D, Henderson D, Harris S (1991) Giant cell arteritis associated with pericarditis and large vessel disease. Aust N Z J Med 21:353–355 10. Pedro-Botet J, Coll J, Lopez MJ, Grau JM (1996) Pericardial effusion and giant cell arteritis. Br J Rheumatol 35:194–195
11. Pattis P, Stockner I, Amor H (2000) Pericardial effusion and aortitis: unusual main manifestations of giant cell arteritis. Acta Med Austriaca 27:83–86 12. Butler JV, Callaghan J, O’Keeffe S, Mulkerrin EC (2001) Giant cell arteritis and pericardial effusion. Ir Med J 94:87–88 13. Valstar MH, Terpstra WF, de Jong RS (2003) Pericardial and pleural effusion in giant cell arteritis. Am J Med 114:708–709 14. Teixera A, Capitaine E, Congy F, Herson S, Cherin P (2003) Myopericarditis during Horton disease. Rev Med Interne 24:189–194 15. Bablekos GD, Michaelides SA, Karachalios GN, Nicolaou IN, Batistatou AK, Charalabopoulos KA (2006) Pericardial involvement as an atypical manifestation of giant cell arteritis. Am J Med Sci 332:198–204 16. Guindon A, Rossi P, Bagneres D et al (2007) Pericarditis: a giant cell arteritis manifestation. Rev Med Interne 28:326–331 17. Moulis G, Sailler L, Astudillo L, Vernet J, Couret B, Arlet P (2010) Pericarditis as the presenting manifestation of giant cell arteritis. Rev Med Interne 31:46–48 18. Blot M, Guépet H, Aubriot-Lorton MH, Pfitzenmeyer P, Manckoundia P (2010) An atypical case of giant cell arteritis (Horton’s disease) associated with facial swelling, confusion, and pericarditis in an elderly woman. J Am Geriatr Soc 58:2040–2041 19. Couturier B, Huyge V, Soyfoo MS (2011) Pericardi tis revealing large vessel vasculitis. ISRN Rheumatol. doi:10.5402/2011/648703 20. Matsue Y, Ohno M, Nagahori W, Suzuki M, Matsumura A, Hashimoto Y (2011) A case of giant cell arteritis with massive pericardial effusion. Heart Vessels 26:562–564 21. Hunder CG, Bloch DA, Michel BA et al (1990) The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 33:1122–1128 22. Zenone T (2006) Fever of unknown origin in adults: evaluation of 144 cases in a non-university hospital. Scand J Infect Dis 38:625–631 23. Zenone T, Puget M (2013) Dry cough is a frequent manifestation of giant cell arteritis. Rheumatol Int 33:2165–2168 24. Ramos A, Laguna P, Cuervas V (1992) Pleural effusion in giant cell arteritis. Ann Intern Med 116:957 25. Garcia-Alfranca F, Solans R, Simenon C et al (1998) Pleural effusion as a form of presentation of temporal arteritis. Br J Rheumatol 37:802–803 26. Marie I, Heliot P, Muir JF et al (2004) Pleural effusion revealing giant cell arteritis. Eur J Intern Med 15:125–127 27. Brucato A, Brambilla G (2002) Polymyalgia rheumatica and pericardial tamponade. Ann Rheum Dis 81:283 28. Malone CB, McCarthy GM (2005) Polymyalgia rheumatica as an unusual cause of pleural and pericardial effusion. J Clin Rheumatol 11:59–60
13
