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Osseous infiltration in a patient with sarcoidosis
European Journal of Internal Medicine 17 (2006) 366 – 367 www.elsevier.com/locate/ejim
Brief report
Osseous infiltration in a patient with sarcoidosis Luisa Sua´rez Alvarez a,*, Luisa Mico´ Giner a, Carmen Alberola Carbonell b, Jose´ Ramo´n Calabuig Alborch a a b
Internal Medicine Department, La Fe Universitary Hospital, Valencia, Spain Pathologic Anatomy Department, La Fe Universitary Hospital, Valencia, Spain
Received 30 August 2005; received in revised form 4 December 2005; accepted 15 December 2005
Abstract A man with lumbar back pain underwent magnetic resonance imaging that showed vertebral and iliac bone lesions. A vertebral biopsy was performed in order to rule out malignancy. Pathology showed non-necrotizing granulomas. The patient had been diagnosed with pulmonary sarcoidosis 20 years earlier and this time the diagnosis made was vertebral sarcoidosis. Osseous infiltration is seen in 13% of patients with sarcoidosis, most of them with long-term disease. Iliac sarcoidosis has seldom been reported, and there are no cases of both iliac and vertebral sarcoidosis in the literature. D 2006 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. Keywords: Sarcoidosis; Bone disease; Magnetic resonance imaging
1. Introduction Sarcoidosis is a granulomatous disorder of unknown etiology that is characterized histopathologically by the presence of noncaseating granulomas in affected organs. Though extrapulmonary sarcoidosis can affect any organ, it most frequently involves the lung. Osseous involvement is uncommon and it can be mistaken for metastatic lesions. We present a case of vertebral and iliac bone involvement in a patient with long-term sarcoidosis who was not receiving treatment at that time.
2. Case report A 65-year-old man was referred to the hospital because of osseous lesions seen on magnetic resonance imaging (MRI). Some weeks earlier, the patients had developed
* Corresponding author. C/Jose´ Ma Haro, N- 51-13 46022 Valencia, Spain. Tel.: +34 963862700x50250. E-mail address: [email protected] (L. Sua´rez Alvarez).
lumbar back pain that radiated to the left leg. The patient had a past history of sarcoidosis; it had been diagnosed 20 years earlier, but he had not been followed or treated in the past 15 years. Physical examination only showed a left L5 radiculopathy. Blood tests revealed the following: glucose 170 mg/dl, triglycerides 201 mg/dl, aspartate aminotransferase 25 UI/l, alanine aminotransferase 43 UI/l, gamma-glutamyl-transpeptidase 75 UI/l and ferritine 167 ng/ml. Ions, urea and creatinine were normal. Hemogram results were: hemoglobin 15.4 mg/dl, hematocrit 44.7%, leukocytes 6500/mm3, platelets 194,000/mm3 and globular sedimentation rate 14/h. Hemostasis was normal. Serum angiotensin-converting enzyme was not measured. Thorax radiography and a computed tomography (CT) scan of the chest showed apical fibrotic lesions and a left parahilar cavity. MRI revealed a herniated disk in L5-S1, and a patchy lesion in the vertebrae T11, T12 and L3 (Fig. 1), and also in the left iliac bone, enhanced following contrast administration. Ziehl stains of several sputum samples, performed in order to rule out tuberculosis, were negative. The left hilar cavity was subsequently attributed to sarcoidosis.
0953-6205/$ - see front matter D 2006 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. doi:10.1016/j.ejim.2005.12.013
L. Sua´rez Alvarez et al. / European Journal of Internal Medicine 17 (2006) 366 – 367
Fig. 1. Vertebral MRI.
These findings were considered highly suspicious for malignancy, so a CT-guided vertebral biopsy was taken. Pathologically, there was no evidence of malignancy or of mycobacterial or fungal infection. It did, however, reveal the existence of non-necrotizing granulomas. A final diagnosis of bone sarcoidosis was made. The patient rested during his admission and his pain gradually decreased.
3. Discussion Sarcoidosis is a multisystemic disease that predominantly affects the lungs and intrathoracic lymph nodes. Bone involvement is seen in up to 13% of patients with sarcoidosis. It is usually a late development [1]. Small bones of the hands and feet are most often affected, but any bone may be involved. Bone disease usually implies a more chronic and severe disease [1,2]. Although bone involvement can be the earliest manifestation of sarcoidosis, it is unusual without infiltrative skin lesions. Vertebral involvement is unusual, though a few cases have been published [3]. Iliac bone lesions are quite uncommon and very few similar cases have been reported in the literature [4]. Almost all patients with osseous lesions are symptomatic and present with radicular back pain during both activity
367
and rest [3]. An evaluation for osseous sarcoidosis should be considered in patients with significant musculoskeletal symptoms, particularly if they also have cutaneous manifestations of sarcoidosis [5]. Granulomatous inflammation can produce osseous lesions that can be detected by radiography as cystic or sclerotic lesions. However, in many cases, plain radiographies are normal and changes can only be seen on MRI or CT scan. The disease most commonly affects the lower dorsal and upper lumbar vertebrae, but the cervical spine may be involved. A CT scan can show either osteosclerotic or osteolitic lesions. MRI may demonstrate single or multiple focal lesions that are hypointense in T1 and hyperintense in T2, enhanced following contrast administration [6]. MRI images are non-specific, and the differential diagnosis includes metastasis, myeloma, lymphoma and granulomatous infection (particularly tuberculosis). Biopsy is recommended in all cases to exclude these other diagnoses. In our patient, the radiographic pulmonary findings also made it necessary to rule out pulmonary tuberculosis, a common disease in our country. Little information is available about the treatment of osseous sarcoidosis. Corticosteroids may control pain and swelling [1]. Cases have been described with good response to methotrexate and hydroxycloroquine, and they can be used in cases of poor response to corticosteroids or recurrences. Symptoms and MRI imaging can be helpful in monitoring the response to therapy. The pathologic MRI abnormalities may become normalized with treatment. In our patient, the pain could have been due to the herniated disc rather than the sarocoidotic lesions, something we interpreted as a casual finding. The pain had improved in our patient by the time he was discharged. He was referred to the Department of Neurosurgery to evaluate the need for surgical treatment of the herniated disk that had probably caused the pain in his leg.
References [1] Wilcoz A, Bharadwaj P, Sharma OP. Bone sarcoidosis. Curr Opin Rheumatol 2000;12:321 – 30. [2] Shorr AF, Murphy FT, Kelly WF. Osseus sarcoidosis: clinical, radiographic, and therapeutic observations. J Clin Rheumatol 1998: 186 – 92. [3] Mangino D, Stover DE. Sarcoidosis presenting as metastatic bone disease. Respiration 2004;71:292 – 4. [4] Andres E, Loth F, Orion B, Marcellin L, Durckel J. Iliac bone defects revealing systemic sarcoidosis. Joint Bone Spine 2001;68:74 – 5. [5] Shorr AF, Murphy FT, Girriland WR, Hnatiuk OW. Osseous disease in patients with pulmonary sarcoidosis and musculoeskeletal symptoms. Respir Med 2000;94:228 – 32. [6] Poyanli A, Poyanli O, Sencer S, Akan K, Sayra KH, Acunas B. Vertebral sarcoidosis: imaging findings. Eur Radiol 2000;10:92 – 4.
Brief report
Osseous infiltration in a patient with sarcoidosis Luisa Sua´rez Alvarez a,*, Luisa Mico´ Giner a, Carmen Alberola Carbonell b, Jose´ Ramo´n Calabuig Alborch a a b
Internal Medicine Department, La Fe Universitary Hospital, Valencia, Spain Pathologic Anatomy Department, La Fe Universitary Hospital, Valencia, Spain
Received 30 August 2005; received in revised form 4 December 2005; accepted 15 December 2005
Abstract A man with lumbar back pain underwent magnetic resonance imaging that showed vertebral and iliac bone lesions. A vertebral biopsy was performed in order to rule out malignancy. Pathology showed non-necrotizing granulomas. The patient had been diagnosed with pulmonary sarcoidosis 20 years earlier and this time the diagnosis made was vertebral sarcoidosis. Osseous infiltration is seen in 13% of patients with sarcoidosis, most of them with long-term disease. Iliac sarcoidosis has seldom been reported, and there are no cases of both iliac and vertebral sarcoidosis in the literature. D 2006 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. Keywords: Sarcoidosis; Bone disease; Magnetic resonance imaging
1. Introduction Sarcoidosis is a granulomatous disorder of unknown etiology that is characterized histopathologically by the presence of noncaseating granulomas in affected organs. Though extrapulmonary sarcoidosis can affect any organ, it most frequently involves the lung. Osseous involvement is uncommon and it can be mistaken for metastatic lesions. We present a case of vertebral and iliac bone involvement in a patient with long-term sarcoidosis who was not receiving treatment at that time.
2. Case report A 65-year-old man was referred to the hospital because of osseous lesions seen on magnetic resonance imaging (MRI). Some weeks earlier, the patients had developed
* Corresponding author. C/Jose´ Ma Haro, N- 51-13 46022 Valencia, Spain. Tel.: +34 963862700x50250. E-mail address: [email protected] (L. Sua´rez Alvarez).
lumbar back pain that radiated to the left leg. The patient had a past history of sarcoidosis; it had been diagnosed 20 years earlier, but he had not been followed or treated in the past 15 years. Physical examination only showed a left L5 radiculopathy. Blood tests revealed the following: glucose 170 mg/dl, triglycerides 201 mg/dl, aspartate aminotransferase 25 UI/l, alanine aminotransferase 43 UI/l, gamma-glutamyl-transpeptidase 75 UI/l and ferritine 167 ng/ml. Ions, urea and creatinine were normal. Hemogram results were: hemoglobin 15.4 mg/dl, hematocrit 44.7%, leukocytes 6500/mm3, platelets 194,000/mm3 and globular sedimentation rate 14/h. Hemostasis was normal. Serum angiotensin-converting enzyme was not measured. Thorax radiography and a computed tomography (CT) scan of the chest showed apical fibrotic lesions and a left parahilar cavity. MRI revealed a herniated disk in L5-S1, and a patchy lesion in the vertebrae T11, T12 and L3 (Fig. 1), and also in the left iliac bone, enhanced following contrast administration. Ziehl stains of several sputum samples, performed in order to rule out tuberculosis, were negative. The left hilar cavity was subsequently attributed to sarcoidosis.
0953-6205/$ - see front matter D 2006 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. doi:10.1016/j.ejim.2005.12.013
L. Sua´rez Alvarez et al. / European Journal of Internal Medicine 17 (2006) 366 – 367
Fig. 1. Vertebral MRI.
These findings were considered highly suspicious for malignancy, so a CT-guided vertebral biopsy was taken. Pathologically, there was no evidence of malignancy or of mycobacterial or fungal infection. It did, however, reveal the existence of non-necrotizing granulomas. A final diagnosis of bone sarcoidosis was made. The patient rested during his admission and his pain gradually decreased.
3. Discussion Sarcoidosis is a multisystemic disease that predominantly affects the lungs and intrathoracic lymph nodes. Bone involvement is seen in up to 13% of patients with sarcoidosis. It is usually a late development [1]. Small bones of the hands and feet are most often affected, but any bone may be involved. Bone disease usually implies a more chronic and severe disease [1,2]. Although bone involvement can be the earliest manifestation of sarcoidosis, it is unusual without infiltrative skin lesions. Vertebral involvement is unusual, though a few cases have been published [3]. Iliac bone lesions are quite uncommon and very few similar cases have been reported in the literature [4]. Almost all patients with osseous lesions are symptomatic and present with radicular back pain during both activity
367
and rest [3]. An evaluation for osseous sarcoidosis should be considered in patients with significant musculoskeletal symptoms, particularly if they also have cutaneous manifestations of sarcoidosis [5]. Granulomatous inflammation can produce osseous lesions that can be detected by radiography as cystic or sclerotic lesions. However, in many cases, plain radiographies are normal and changes can only be seen on MRI or CT scan. The disease most commonly affects the lower dorsal and upper lumbar vertebrae, but the cervical spine may be involved. A CT scan can show either osteosclerotic or osteolitic lesions. MRI may demonstrate single or multiple focal lesions that are hypointense in T1 and hyperintense in T2, enhanced following contrast administration [6]. MRI images are non-specific, and the differential diagnosis includes metastasis, myeloma, lymphoma and granulomatous infection (particularly tuberculosis). Biopsy is recommended in all cases to exclude these other diagnoses. In our patient, the radiographic pulmonary findings also made it necessary to rule out pulmonary tuberculosis, a common disease in our country. Little information is available about the treatment of osseous sarcoidosis. Corticosteroids may control pain and swelling [1]. Cases have been described with good response to methotrexate and hydroxycloroquine, and they can be used in cases of poor response to corticosteroids or recurrences. Symptoms and MRI imaging can be helpful in monitoring the response to therapy. The pathologic MRI abnormalities may become normalized with treatment. In our patient, the pain could have been due to the herniated disc rather than the sarocoidotic lesions, something we interpreted as a casual finding. The pain had improved in our patient by the time he was discharged. He was referred to the Department of Neurosurgery to evaluate the need for surgical treatment of the herniated disk that had probably caused the pain in his leg.
References [1] Wilcoz A, Bharadwaj P, Sharma OP. Bone sarcoidosis. Curr Opin Rheumatol 2000;12:321 – 30. [2] Shorr AF, Murphy FT, Kelly WF. Osseus sarcoidosis: clinical, radiographic, and therapeutic observations. J Clin Rheumatol 1998: 186 – 92. [3] Mangino D, Stover DE. Sarcoidosis presenting as metastatic bone disease. Respiration 2004;71:292 – 4. [4] Andres E, Loth F, Orion B, Marcellin L, Durckel J. Iliac bone defects revealing systemic sarcoidosis. Joint Bone Spine 2001;68:74 – 5. [5] Shorr AF, Murphy FT, Girriland WR, Hnatiuk OW. Osseous disease in patients with pulmonary sarcoidosis and musculoeskeletal symptoms. Respir Med 2000;94:228 – 32. [6] Poyanli A, Poyanli O, Sencer S, Akan K, Sayra KH, Acunas B. Vertebral sarcoidosis: imaging findings. Eur Radiol 2000;10:92 – 4.