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AL amyloidosis with temporal artery involvement simulates giant-cell arteritis
Joint Bone Spine 79 (2012) 195–197
Available online at
www.sciencedirect.com
Case report
AL amyloidosis with temporal artery involvement simulates giant-cell arteritis Alexandra Audemard a,1 , Jonathan Boutemy a,d,1 , Franc¸oise Galateau-Salle b,d,1 , Margaret Macro c,1 , Boris Bienvenu a,d,1,∗ a
Department of Internal Medicine, CHU de Caen, 14000 Caen, France Department of Pathology, CHU de Caen, 14000 Caen, France Department of Haematology, CHU de Caen, 14000 Caen, France d Université de Caen Basse-Normandie, UFR de Médecine, 14000 Caen, France b c
a r t i c l e
i n f o
Article history: Accepted 28 September 2011 Available online 28 December 2011 Keywords: AL amyloidosis Primary amyloidosis Giant-cell arteritis Temporal arterial biopsy
a b s t r a c t Light-chain (AL) amyloidosis may present with features suggesting vasculitis, including giant-cell arteritis (GCA). We describe a case of an 80-year-old man, who initially presented with bilateral jaw claudication, bi-temporal headache and arthralgia, however a temporal-artery biopsy then revealed AL amyloidosis. A diagnosis of AL amyloidosis complicating multiple myelome simulates GCA and polymyalgia rheumatica was established. The patient was successfully treated with melphalan and dexamethasone: the free kappa light chains decreased, the patient’s jaw claudication and headache disappeared. Then we discuss similarities between GCA and AL amyloidosis and potential confusion in diagnosis. We suggest that, in patients with clinical features of GCA without any temporal-artery typical findings, specimens are stained with Congo red, which then results in a different diagnosis and treatment. © 2011 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.
Giant-cell arteritis (GCA), or temporal arteritis, is a granulomatous vasculitis that affects large- and medium-caliber arteries and has an unknown etiology. Classical symptoms of the disease are headache, jaw claudication, and sudden loss of vision. Lightchain (AL) amyloidosis, or primary amyloidosis, is a plasma-cell dyscrasia associated with an asymptomatic myeloma in 40% of cases. It is characterized by deposition of monoclonal light-chain variable-region fragments. The insoluble polymeric protein fibrils may involve any tissue or organ, except for the central nervous system. They mainly involve the heart, kidneys, and peripheral nerves. AL amyloidosis of the vascular system is frequent and usually occurs in small-caliber vessels. Localization to medium or large vessels is unusual. Coronary or hepatic-artery involvement has been described. Herein, we describe a case of an 80-year-old man who presented initially with GCA and polymyalgia rheumatica (PMR); however, a temporal artery biopsy then revealed AL amyloidosis. We have reviewed similar case reports from the literature. 1. Case report An 80-year-old man was transferred to our hospital with a 6month history of weight loss, weakness, and decreased appetite. His medical history included hypertension. Six months before
∗ Corresponding author. E-mail address: [email protected] (B. Bienvenu). 1 All authors who contributed substantially to the manuscript have agreed with the data presented and its interpretations, and have given permission for its submission.
evaluation, he developed bilateral jaw claudication, bi-temporal headache, myalgia, arthralgia of his shoulder, and acral paresthesia. On examination, the patient appeared well and his vital signs were normal. The temporal arteries were noted to be prominent (Fig. 1), with tenderness on palpation and decreased pulsation. The patient presented with “shoulder pad sign”, macroglossia, buccal dryness, and acral hypoesthesia with areflexia. Results from a complete blood-cell count, renal function, C-reactive protein, and calcium levels were all within normal ranges. Ultrasonographic evaluation of the temporal artery revealed a “halo sign” suggestive of GCA. A temporal-artery biopsy was performed. Hematoxylin and eosin–saffron staining of a temporal-artery biopsy showed concentric intimal thickening. Elastolysis, with disruption of the internal lamina, was observed. Deposition of large amorphous, eosinophilic material, suggestive of amyloid deposits in the media of the temporal artery, as well as the small vessels of the adventitia were observed (Fig. 2). There were no multinucleated giant-cells. Immunohistochemical stains for lambda and kappa chains revealed that the eosinophilic deposits were kappa positive (Fig. 3) and lambda negative. Further analysis with anti-serum amyloid A protein was negative, and was positive for the anti-serum P component. Further investigations were performed on the basis of a diagnosis of AL amyloidosis. Electrophoresis did not reveal a monoclonal spike, but elevated free kappa light chains (354 mg/L) with a kappa/lambda ratio of 47:1. A bone-marrow aspiration showed 15% atypical plasma cells, which revealed asymptomatic multiple myeloma. Skeletal Xrays were normal. An echocardiogram displayed concentrically hyperechogenic and thickened ventricles, which were compatible
1297-319X/$ – see front matter © 2011 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. doi:10.1016/j.jbspin.2011.09.007
196
A. Audemard et al. / Joint Bone Spine 79 (2012) 195–197
Fig. 3. Kappa-positive amyloid deposits.
Fig. 1. Prominent temporal artery.
with cardiac amyloidosis. Troponin and brain natriuretic peptide were normal. An electro-diagnostic study showed axonal sensory polyneuropathy. The patient was treated with melphalan and dexamethasone. One month later, the free kappa light chains had decreased to 250 mg/L and the patient’s jaw claudication and headache had disappeared. After four cycles with mephalan and dexamethasone, the free light chains and the thickened ventricles, measured by echocardiogram, had increased. A second-line treatment of bortezomib was started. 2. Discussion We report an original case of AL amyloidosis involving the temporal artery, which presented with symptoms of GCA and polymyalgia rheumatica.
Fig. 2. Deposition of amorphous, eosinophilic, hyaline material.
The criteria for the classification of GCA, from the American College of Rheumatology (ACR), includes age over 50 years, erythrocyte-sedimentation rate up to 50 mm, superficial temporalartery tenderness, temporal headache, and GCA, as seen in a temporal-artery biopsy. This patient fulfilled four of the five criteria. Three or more factors are considered to be highly accurate (sensitivity 93.5%, specificity 91.2%) in distinguishing GCA from other forms of vasculitis, although it does not provide a diagnosis score. The sensibility and specificity of the ACR criteria depend upon the population studied. This case illustrates that physicians can over-diagnose GCA, especially because the ACR criteria lack specificity in the general population. A temporal-artery biopsy is the gold standard for diagnosis of GCA. A review of the literature showed 14 cases of AL amyloidosis presenting as GCA, whereas temporal-artery biopsies then confirmed AL amyloidosis in these cases [1–14]. Two of the diagnoses were made after re-staining with Congo red, suggesting that amyloid deposits can be difficult to identify. Because of the frequency of negative temporal-artery biopsies in GCA, we can hypothesize that some patients present with AL amyloidosis instead of GCA. It would be interesting to perform a second study on negative temporalartery biopsies in patients with temporal syndrome, to detect AL amyloidosis. One case of an association between AL amyloidosis and GCA has been reported [4], a temporal biopsy revealed multinucleated giant cells that were engulfing amyloid deposits. We can hypothesize that the giant cells may have been a reactive response to the amyloid deposits rather than the two entities had occurred simultaneously. In another case [8], a few giant cells were detected surrounded by an amyloid deposit. In GCA, giant-cells are localized to the internal elastic lamina whereas, in AL amyloidosis, they are located next to the amyloid deposit. In addition to the confusion between the clinical symptoms of GCA an AL amyloidosis, histologic findings in patients with amyloidosis can mimic a giant-cell lesion. We could expect more incidences of frequent amyloid cardiopathy in patients with temporal-artery involvement. However, only four of the fourteen patients (28%) had a specific cardiopathy. According to the reported literature, 30 to 50% of patients had cardiac involvement in AL amyloidosis. Four of the fourteen patients had concomitant leg or calf claudication, which could be linked to amyloidosis, suggesting diffuse vasculature involvement. Meretoja and Tarkkanen [13] suggest that amyloid deposits may play a role in the pathogenesis of GCA, and described ten patients with temporal arteritis and amyloidosis of the internal elastic lamina. Muckle [14] later claimed that any association between the
A. Audemard et al. / Joint Bone Spine 79 (2012) 195–197
occurrence of GCA and senile amyloidosis of the internal lamina of the temporal artery was associated incidentally with old age. Because the rate of senile amyloidosis progresses to 100% in the ninth decades of life [14], it is very important to routinely assess immunohistochemistry to avoid misdiagnosis of AL amyloidosis in the presence of senile amyloid deposits, although senile amyloid deposits are generally asymptomatic. AL amyloidosis is composed of variable (V)-domain immunoglobulin light chains. Amyloid fibrils share a structural B-pleated sheet conformation in common, which confers them their unique staining properties. However, the mechanism of fibril formation and tissue toxicity remains controversial. Factors that contribute to fibrillogenesis include variable or unstable protein structure, extensive beta-sheet conformation of the precursor protein, proteolytic processing of the precursor protein, and association with components of the serum or extracellular matrix. Both kappa and lambda light chains have been identified in AL amyloid fibrils: lambda chains predominate, and there is a kappa to lambda ratio frequency of 1:2. Two V lambda gene components 6 and 3) equally contribute to encoding 42% of the amyloidogenic lambda light chains, and appear to have unique structural properties that predispose them to fibril formation. Comenzo et al. documented that lambda-6 light chains are more likely to be found in patients with dominant renal involvement, raising the possibility that the diverse organ tropism of amyloid light chains may be influenced by V lambda gene usage [15]. If this is the case, the V kappa chain may have the ability to cause deposition and be able to cause deposition within the vasculature. Investigations in experimental mice models have demonstrated that amyloids preferentially accumulate around leaky areas of vasculature. Examples of amyloidosis affecting the vasculature have been described in pre-existing vascular anomalies, e.g., localized amyloidosis was found in a gastric arteriovenous malformation. In our case, we hypothesize that hypertension contributed to the temporal end-artery damage (intimal fibrosis) and favored medial amyloid deposits. This suggests that the deposition mechanism for light chains was endoluminal. However, amyloid deposition is still not fully understood. It may arise from the vaso vasorum in the adventitious temporal artery, as testified by predominant vasa vasorum involvement in the outer layer of the media.
197
We recommend that, in patients with clinical features of GCA without any typical biopsy findings, specimens are stained with Congo red as amyloid deposition may be the cause of the symptoms, which then results in a different diagnosis and treatment: corticosteroid for GCA and chemotherapy for AL amyloidosis. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Hamidou M, Buzelin F, Rojouan J, et al. Temporal artery syndrome in amyloidosis AL associated with myeloma. Rev Med Interne 1991;12:306–8. [2] Li MC, Chou G, Chen JT, et al. Amyloidosis of medium-sized arteries presenting as perioral mass: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:463–6. [3] Nguyen TB, Auroy S, Baviera E, et al. IgG kappa monoclonal gammopathy complicated by AL amyloidosis with symptomatic involvement of the temporal artery. Rev Med Interne 1996;17:502. [4] Estrada A, Stenzel TT, Burchette JL, et al. Multiple myeloma-associated amyloidosis and giant cell arteritis. Arthritis Rheum 1998;41:1312–7. [5] Churchill CH, Abril A, Krishna M, et al. Jaw claudication in primary amyloidosis: unusual presentation of a rare disease. J Rheumatol 2003;30:2283–6. [6] Rao JK, Allen NB. Primary systemic amyloidosis masquerading as giant cell arteritis. Case report and review of the literature. Arthritis Rheum 1993;36:422–5. [7] Salvarani C, Gabriel SE, Gertz MA, et al. Primary systemic amyloidosis presenting as giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum 1994;37:1621–6. [8] Tron AM, Piperno M, Bouvier R, et al. Primary AL-type amyloidosis disclosed by Horton disease. Rev Med Interne 1995;16:850–3. [9] Taillan B, Fuzibet JG, Vinti H, et al. AL amyloid deposits in temporal artery mimicking giant cell arteritis. Clin Rheumatol 1990;9:256. [10] Gertz MA, Kyle RA, Griffing WL, et al. Jaw claudication in primary systemic amyloidosis. Medicine (Baltimore) 1986;65:173–9. [11] Ing EB, Woolf IZ, Younge BR, et al. Systemic amyloidosis with temporal artery involvement mimicking temporal arteritis. Ophthalmic Surg Lasers 1997;28:328–31. [12] Rodon P, Friocourt P, Blanchet S, et al. Temporal artery involvement revealing AL amyloidosis and IgD monoclonal gammopathy. J Rheumatol 1996;23:189–90. [13] Meretoja J, Tarkkanen A. Amyloid deposits of internal elastic lamina in temporal arteritis. Ophtalmologica 1975;170:337–44. [14] Muckle TJ. Giant cell inflammation compared with amyloidosis of the internal elastic lamina in temporal arteries. Arthritis Rheum 1988;31:1186–9. [15] Comenzo RL, Zhang Y, Martinez C, et al. The tropism of organ involvement in primary systemic amyloidosis: contributions of Ig V(L) germ line gene use and clonal plasma cell burden. Blood 2001;98:714–20.
Available online at
www.sciencedirect.com
Case report
AL amyloidosis with temporal artery involvement simulates giant-cell arteritis Alexandra Audemard a,1 , Jonathan Boutemy a,d,1 , Franc¸oise Galateau-Salle b,d,1 , Margaret Macro c,1 , Boris Bienvenu a,d,1,∗ a
Department of Internal Medicine, CHU de Caen, 14000 Caen, France Department of Pathology, CHU de Caen, 14000 Caen, France Department of Haematology, CHU de Caen, 14000 Caen, France d Université de Caen Basse-Normandie, UFR de Médecine, 14000 Caen, France b c
a r t i c l e
i n f o
Article history: Accepted 28 September 2011 Available online 28 December 2011 Keywords: AL amyloidosis Primary amyloidosis Giant-cell arteritis Temporal arterial biopsy
a b s t r a c t Light-chain (AL) amyloidosis may present with features suggesting vasculitis, including giant-cell arteritis (GCA). We describe a case of an 80-year-old man, who initially presented with bilateral jaw claudication, bi-temporal headache and arthralgia, however a temporal-artery biopsy then revealed AL amyloidosis. A diagnosis of AL amyloidosis complicating multiple myelome simulates GCA and polymyalgia rheumatica was established. The patient was successfully treated with melphalan and dexamethasone: the free kappa light chains decreased, the patient’s jaw claudication and headache disappeared. Then we discuss similarities between GCA and AL amyloidosis and potential confusion in diagnosis. We suggest that, in patients with clinical features of GCA without any temporal-artery typical findings, specimens are stained with Congo red, which then results in a different diagnosis and treatment. © 2011 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.
Giant-cell arteritis (GCA), or temporal arteritis, is a granulomatous vasculitis that affects large- and medium-caliber arteries and has an unknown etiology. Classical symptoms of the disease are headache, jaw claudication, and sudden loss of vision. Lightchain (AL) amyloidosis, or primary amyloidosis, is a plasma-cell dyscrasia associated with an asymptomatic myeloma in 40% of cases. It is characterized by deposition of monoclonal light-chain variable-region fragments. The insoluble polymeric protein fibrils may involve any tissue or organ, except for the central nervous system. They mainly involve the heart, kidneys, and peripheral nerves. AL amyloidosis of the vascular system is frequent and usually occurs in small-caliber vessels. Localization to medium or large vessels is unusual. Coronary or hepatic-artery involvement has been described. Herein, we describe a case of an 80-year-old man who presented initially with GCA and polymyalgia rheumatica (PMR); however, a temporal artery biopsy then revealed AL amyloidosis. We have reviewed similar case reports from the literature. 1. Case report An 80-year-old man was transferred to our hospital with a 6month history of weight loss, weakness, and decreased appetite. His medical history included hypertension. Six months before
∗ Corresponding author. E-mail address: [email protected] (B. Bienvenu). 1 All authors who contributed substantially to the manuscript have agreed with the data presented and its interpretations, and have given permission for its submission.
evaluation, he developed bilateral jaw claudication, bi-temporal headache, myalgia, arthralgia of his shoulder, and acral paresthesia. On examination, the patient appeared well and his vital signs were normal. The temporal arteries were noted to be prominent (Fig. 1), with tenderness on palpation and decreased pulsation. The patient presented with “shoulder pad sign”, macroglossia, buccal dryness, and acral hypoesthesia with areflexia. Results from a complete blood-cell count, renal function, C-reactive protein, and calcium levels were all within normal ranges. Ultrasonographic evaluation of the temporal artery revealed a “halo sign” suggestive of GCA. A temporal-artery biopsy was performed. Hematoxylin and eosin–saffron staining of a temporal-artery biopsy showed concentric intimal thickening. Elastolysis, with disruption of the internal lamina, was observed. Deposition of large amorphous, eosinophilic material, suggestive of amyloid deposits in the media of the temporal artery, as well as the small vessels of the adventitia were observed (Fig. 2). There were no multinucleated giant-cells. Immunohistochemical stains for lambda and kappa chains revealed that the eosinophilic deposits were kappa positive (Fig. 3) and lambda negative. Further analysis with anti-serum amyloid A protein was negative, and was positive for the anti-serum P component. Further investigations were performed on the basis of a diagnosis of AL amyloidosis. Electrophoresis did not reveal a monoclonal spike, but elevated free kappa light chains (354 mg/L) with a kappa/lambda ratio of 47:1. A bone-marrow aspiration showed 15% atypical plasma cells, which revealed asymptomatic multiple myeloma. Skeletal Xrays were normal. An echocardiogram displayed concentrically hyperechogenic and thickened ventricles, which were compatible
1297-319X/$ – see front matter © 2011 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. doi:10.1016/j.jbspin.2011.09.007
196
A. Audemard et al. / Joint Bone Spine 79 (2012) 195–197
Fig. 3. Kappa-positive amyloid deposits.
Fig. 1. Prominent temporal artery.
with cardiac amyloidosis. Troponin and brain natriuretic peptide were normal. An electro-diagnostic study showed axonal sensory polyneuropathy. The patient was treated with melphalan and dexamethasone. One month later, the free kappa light chains had decreased to 250 mg/L and the patient’s jaw claudication and headache had disappeared. After four cycles with mephalan and dexamethasone, the free light chains and the thickened ventricles, measured by echocardiogram, had increased. A second-line treatment of bortezomib was started. 2. Discussion We report an original case of AL amyloidosis involving the temporal artery, which presented with symptoms of GCA and polymyalgia rheumatica.
Fig. 2. Deposition of amorphous, eosinophilic, hyaline material.
The criteria for the classification of GCA, from the American College of Rheumatology (ACR), includes age over 50 years, erythrocyte-sedimentation rate up to 50 mm, superficial temporalartery tenderness, temporal headache, and GCA, as seen in a temporal-artery biopsy. This patient fulfilled four of the five criteria. Three or more factors are considered to be highly accurate (sensitivity 93.5%, specificity 91.2%) in distinguishing GCA from other forms of vasculitis, although it does not provide a diagnosis score. The sensibility and specificity of the ACR criteria depend upon the population studied. This case illustrates that physicians can over-diagnose GCA, especially because the ACR criteria lack specificity in the general population. A temporal-artery biopsy is the gold standard for diagnosis of GCA. A review of the literature showed 14 cases of AL amyloidosis presenting as GCA, whereas temporal-artery biopsies then confirmed AL amyloidosis in these cases [1–14]. Two of the diagnoses were made after re-staining with Congo red, suggesting that amyloid deposits can be difficult to identify. Because of the frequency of negative temporal-artery biopsies in GCA, we can hypothesize that some patients present with AL amyloidosis instead of GCA. It would be interesting to perform a second study on negative temporalartery biopsies in patients with temporal syndrome, to detect AL amyloidosis. One case of an association between AL amyloidosis and GCA has been reported [4], a temporal biopsy revealed multinucleated giant cells that were engulfing amyloid deposits. We can hypothesize that the giant cells may have been a reactive response to the amyloid deposits rather than the two entities had occurred simultaneously. In another case [8], a few giant cells were detected surrounded by an amyloid deposit. In GCA, giant-cells are localized to the internal elastic lamina whereas, in AL amyloidosis, they are located next to the amyloid deposit. In addition to the confusion between the clinical symptoms of GCA an AL amyloidosis, histologic findings in patients with amyloidosis can mimic a giant-cell lesion. We could expect more incidences of frequent amyloid cardiopathy in patients with temporal-artery involvement. However, only four of the fourteen patients (28%) had a specific cardiopathy. According to the reported literature, 30 to 50% of patients had cardiac involvement in AL amyloidosis. Four of the fourteen patients had concomitant leg or calf claudication, which could be linked to amyloidosis, suggesting diffuse vasculature involvement. Meretoja and Tarkkanen [13] suggest that amyloid deposits may play a role in the pathogenesis of GCA, and described ten patients with temporal arteritis and amyloidosis of the internal elastic lamina. Muckle [14] later claimed that any association between the
A. Audemard et al. / Joint Bone Spine 79 (2012) 195–197
occurrence of GCA and senile amyloidosis of the internal lamina of the temporal artery was associated incidentally with old age. Because the rate of senile amyloidosis progresses to 100% in the ninth decades of life [14], it is very important to routinely assess immunohistochemistry to avoid misdiagnosis of AL amyloidosis in the presence of senile amyloid deposits, although senile amyloid deposits are generally asymptomatic. AL amyloidosis is composed of variable (V)-domain immunoglobulin light chains. Amyloid fibrils share a structural B-pleated sheet conformation in common, which confers them their unique staining properties. However, the mechanism of fibril formation and tissue toxicity remains controversial. Factors that contribute to fibrillogenesis include variable or unstable protein structure, extensive beta-sheet conformation of the precursor protein, proteolytic processing of the precursor protein, and association with components of the serum or extracellular matrix. Both kappa and lambda light chains have been identified in AL amyloid fibrils: lambda chains predominate, and there is a kappa to lambda ratio frequency of 1:2. Two V lambda gene components 6 and 3) equally contribute to encoding 42% of the amyloidogenic lambda light chains, and appear to have unique structural properties that predispose them to fibril formation. Comenzo et al. documented that lambda-6 light chains are more likely to be found in patients with dominant renal involvement, raising the possibility that the diverse organ tropism of amyloid light chains may be influenced by V lambda gene usage [15]. If this is the case, the V kappa chain may have the ability to cause deposition and be able to cause deposition within the vasculature. Investigations in experimental mice models have demonstrated that amyloids preferentially accumulate around leaky areas of vasculature. Examples of amyloidosis affecting the vasculature have been described in pre-existing vascular anomalies, e.g., localized amyloidosis was found in a gastric arteriovenous malformation. In our case, we hypothesize that hypertension contributed to the temporal end-artery damage (intimal fibrosis) and favored medial amyloid deposits. This suggests that the deposition mechanism for light chains was endoluminal. However, amyloid deposition is still not fully understood. It may arise from the vaso vasorum in the adventitious temporal artery, as testified by predominant vasa vasorum involvement in the outer layer of the media.
197
We recommend that, in patients with clinical features of GCA without any typical biopsy findings, specimens are stained with Congo red as amyloid deposition may be the cause of the symptoms, which then results in a different diagnosis and treatment: corticosteroid for GCA and chemotherapy for AL amyloidosis. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Hamidou M, Buzelin F, Rojouan J, et al. Temporal artery syndrome in amyloidosis AL associated with myeloma. Rev Med Interne 1991;12:306–8. [2] Li MC, Chou G, Chen JT, et al. Amyloidosis of medium-sized arteries presenting as perioral mass: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:463–6. [3] Nguyen TB, Auroy S, Baviera E, et al. IgG kappa monoclonal gammopathy complicated by AL amyloidosis with symptomatic involvement of the temporal artery. Rev Med Interne 1996;17:502. [4] Estrada A, Stenzel TT, Burchette JL, et al. Multiple myeloma-associated amyloidosis and giant cell arteritis. Arthritis Rheum 1998;41:1312–7. [5] Churchill CH, Abril A, Krishna M, et al. Jaw claudication in primary amyloidosis: unusual presentation of a rare disease. J Rheumatol 2003;30:2283–6. [6] Rao JK, Allen NB. Primary systemic amyloidosis masquerading as giant cell arteritis. Case report and review of the literature. Arthritis Rheum 1993;36:422–5. [7] Salvarani C, Gabriel SE, Gertz MA, et al. Primary systemic amyloidosis presenting as giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum 1994;37:1621–6. [8] Tron AM, Piperno M, Bouvier R, et al. Primary AL-type amyloidosis disclosed by Horton disease. Rev Med Interne 1995;16:850–3. [9] Taillan B, Fuzibet JG, Vinti H, et al. AL amyloid deposits in temporal artery mimicking giant cell arteritis. Clin Rheumatol 1990;9:256. [10] Gertz MA, Kyle RA, Griffing WL, et al. Jaw claudication in primary systemic amyloidosis. Medicine (Baltimore) 1986;65:173–9. [11] Ing EB, Woolf IZ, Younge BR, et al. Systemic amyloidosis with temporal artery involvement mimicking temporal arteritis. Ophthalmic Surg Lasers 1997;28:328–31. [12] Rodon P, Friocourt P, Blanchet S, et al. Temporal artery involvement revealing AL amyloidosis and IgD monoclonal gammopathy. J Rheumatol 1996;23:189–90. [13] Meretoja J, Tarkkanen A. Amyloid deposits of internal elastic lamina in temporal arteritis. Ophtalmologica 1975;170:337–44. [14] Muckle TJ. Giant cell inflammation compared with amyloidosis of the internal elastic lamina in temporal arteries. Arthritis Rheum 1988;31:1186–9. [15] Comenzo RL, Zhang Y, Martinez C, et al. The tropism of organ involvement in primary systemic amyloidosis: contributions of Ig V(L) germ line gene use and clonal plasma cell burden. Blood 2001;98:714–20.