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Jaw Claudication in Primary Amyloidosis: Unusual Presentation of a Rare Disease CHRISTOPHER H

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Jaw Claudication in Primary Amyloidosis: Unusual Presentation of a Rare Disease CHRISTOPHER H Case Report Jaw Claudication in Primary Amyloidosis: Unusual Presentation of a Rare Disease CHRISTOPHER H. CHURCHILL, ANDYABRIL, MURLI KRISHNA, MARK L. CALLMAN, and WILLIAM W. GINSBURG ABSTRACT. We describe 2 patients with temporal artery biopsy-proven amyloidosis presenting with symptoms of jaw claudication, visual disturbance, and proximal muscle stiffness suggestive of giant cell arteritis (GCA) and polymyalgia rheumatica. At the onset of disease, neither patient had other char- acteristic symptoms to suggest primary amyloid. We point out similarities between GCA and primary amyloid that can lead to confusion in diagnosis. (J Rheumatol 2003;30:2283–6) Key Indexing Terms: GIANT CELL ARTERITIS POLYMYALGIA RHEUMATICA PRIMARY AMYLOIDOSIS JAW CLAUDICATION Primary amyloid is a rare disease that characteristically he had been prescribed 10 mg prednisone with no benefit. Within the month presents with findings that include carpal tunnel syndrome, prior to his visit while taking prednisone 10 mg he noted some swelling of the right wrist without symptoms of carpal tunnel syndrome. He also devel- congestive heart failure, nephrotic syndrome, peripheral oped jaw claudication and 2 episodes of diplopia. He had no headache, 1 neuropathy, and orthostatic hypotension . Our 2 patients scalp tenderness, or fever. His history included long-standing hypertension presented with symptoms of jaw claudication and visual and atherosclerotic cardiovascular disease, with 5-vessel bypass surgery in disturbance suggestive of giant cell arteritis (GCA) and had 1998 and a carotid endarterectomy in 1999. polymyalgia rheumatica (PMR)-like symptoms. Both had Examination revealed prominent temporal arteries with normal pulses and no tenderness. Cardiac, pulmonary, and abdominal examination were anemia and an elevated erythrocyte sedimentation rate unremarkable. There was no macroglossia or purpura. There was some (ESR). Neither patient had typical symptoms of primary slight swelling of the right wrist, with no other evidence of joint swelling. amyloid at onset of their disease. The diagnosis was not Laboratory studies included hemoglobin of 10.3 g/dl, ESR 38 mm/h, considered until a temporal artery biopsy revealed amyloid creatinine 1.3 mg/dl, urinalysis with grade 2 protein, and normal serum deposition in one patient; in the second patient worsening protein electrophoresis. A presumptive diagnosis of GCA was made, and because of the renal function and proteinuria led to a renal biopsy that diplopia, treatment with prednisone 60 mg daily was initiated. A temporal confirmed amyloid deposition. The temporal artery biopsy artery biopsy 2 days later revealed extensive deposits that were Congo red was also positive for amyloid. positive (Figures 1–3). A slight mononuclear cell infiltrate was noted. In this report, we emphasize that symptoms suggestive of Further investigation revealed a kappa light-chain on serum immunoelec- GCA can also be seen in primary amyloid. If a temporal artery trophoresis. Staining of the temporal artery also revealed kappa light-chain and a bone marrow biopsy had 20% to 30% atypical plasma cells, which biopsy is negative for granulomatous inflammation, a Congo stained for monoclonal kappa light-chain. There was no amyloid deposi- red stain should be performed to investigate for amyloid. tion. A diagnosis of multiple myeloma with associated primary amyloid was made. He did have a partial response to the prednisone, with decreased CASE REPORTS stiffness, increased energy, and improvement of his jaw claudication. Case 1. A 76-year-old man was referred for evaluation of muscle pain and Subsequently the prednisone was tapered and melphalan was to be started. stiffness, weight loss, and malaise over the previous 2 months. Laboratory Case 2. A 64-year-old man was referred for evaluation of shoulder pain and studies obtained by his primary physician included an elevated ESR of 70 stiffness, jaw pain, and elevated ESR. He had started experiencing symp- mm/h and a normocytic, normochromic anemia with low serum iron and toms 6 months previously. He initially noted morning stiffness of the shoul- normal ferritin. The symptoms and laboratory findings suggested PMR, and ders, which lasted one hour, followed by pain that lasted all day. He also experienced weight loss, fatigue, transient episodes of visual loss that From the Divisions of Rheumatology and Pathology, Mayo Clinic, lasted 4–5 minutes, and jaw claudication. He had also recently developed a Jacksonville, Florida; and Premier Medical Group, Port Charlotte, goiter, which had been growing rapidly and had been evaluated by Florida, USA. endocrinology. Outside records revealed elevated sedimentation rates C.H. Churchill, Medical Student; A. Abril, MD, Instructor of Internal ranging from 40 to 70 mm/h. During the initial evaluation the only signifi- Medicine, Division of Rheumatology; M. Krishna, MD, Assistant cant finding on examination was a large goiter. ESR was 61 mm/h; he had Professor of Pathology; W.W. Ginsburg, MD, Associate Professor of normocytic anemia with hemoglobin of 10.7 g/dl, and the serum creatinine Medicine, Division of Rheumatology, Mayo Clinic Jacksonville; M.L. was 1.6 mg/dl. A serum protein electrophoresis was normal. Callman, MD, Internist, Premier Medical Group. With a presumptive diagnosis of temporal arteritis a temporal artery Address reprint requests to Dr. W.W. Ginsburg, Mayo Clinic Jacksonville, biopsy was obtained and it was negative for GCA. The goiter was excised 4500 San Pablo Road, Jacksonville, FL 32224. later and revealed adenomatous hyperplasia. Congo red stains were not Submitted November 8, 2002; revision accepted February 24, 2003. initially obtained on the biopsies. Personal, non-commercial use only. The Journal of Rheumatology Copyright © 2003. All rights reserved. Churchill, et al: Jaw claudication in amyloidosis 2283 Downloaded on September 26, 2021 from www.jrheum.org COLOUR Figure 1. Hematoxylin and eosin stain showing eosinophilic deposits within the arterial wall (original magnification ×40). Figure 2. Congo red stain highlighting amyloid deposits (original magnification ×40). Figure 3. Blue-green birefringence seen under polarized light (original magnifica- tion ×100). Personal, non-commercial use only. The Journal of Rheumatology Copyright © 2003. All rights reserved. 2284 The Journal of Rheumatology 2003; 30:10 Downloaded on September 26, 2021 from www.jrheum.org He was empirically started on prednisone initially 10 mg/day and then and 5 had calf claudication. The median age of patients was increased to 20 mg/day, with only mild improvement of the general symp- 60.5 years. Weakness and fatigue were experienced by 59%. toms. Over the next 8 months he developed left side chest and arm pain; Nine of the 22 patients had vascular ischemia or muscular cardiac catheterization was performed and there were no coronary artery lesions. He then developed worsening renal function indicated by increased pain as the presenting manifestation. Five of 22 patients creatinine concentration and proteinuria; a renal biopsy revealed amyloid. were initially misdiagnosed. Other, more characteristic A lambda light-chain was detected on serum and urine immunoelec- symptoms of AL were present including 9 patients with trophoresis. A bone marrow biopsy revealed a monoclonal lambda plasma carpal tunnel syndrome, 6 with nephrotic syndrome, 6 with cell proliferation without amyloid deposits. A subcutaneous fat aspiration congestive heart failure, 2 with peripheral neuropathy, and revealed amyloid deposits. We went back to the previous temporal artery and thyroid biopsies and they stained positive for amyloid with Congo red. one with orthostatic hypotension. Three of the 22 patients The patient was seen by a hematology specialist; therapy was planned with had none of the more common manifestations6. high dose melphalan and peripheral blood stem cell transplantation. There have been other case reports documenting the confusion between GCA/PMR and AL, when AL presented DISCUSSION with atypical manifestations of claudication and muscle pain We describe 2 patients who presented with classic jaw clau- and stiffness. In one report a patient had symptoms sugges- dication and laboratory evidence supportive of GCA/PMR. tive of GCA including temporal headaches, blurred vision, Jaw claudication is considered a highly specific clinical jaw and right leg claudication, and proximal muscle weak- manifestation of GCA. It is also a relatively common mani- ness. No inflammation was found on temporal artery biopsy, festation of GCA, and in one series of 100 consecutive and a soleus muscle and nerve biopsy stained positive for patients 45% experienced jaw claudication2. Not until a amyloid. Restaining the temporal artery biopsy also temporal artery biopsy in Case 1 and a renal biopsy in Case revealed amyloid. The patient did receive high dose methyl- 2 revealed amyloid was this diagnosis considered. Neither prednisolone before the diagnosis of AL was apparent, and patient had other common clinical symptoms of primary had marked improvement of visual symptoms, headaches, systemic amyloid such as nephrotic syndrome, congestive and proximal muscle weakness. After the diagnosis steroids heart failure, carpal tunnel syndrome, peripheral were tapered to 20 mg prednisone, but at this dose the neuropathy, or orthostatic hypotension at the onset of patient had recurrence of weakness and pain and died within disease. 8 months of diagnosis7. Amyloidosis is a rare systemic disease caused by deposi-
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