Osseous sarcoidosis: Clinical characteristics, treatment, and outcomes—Experience from a large, academic hospital

doi:10.1016/j.semarthrit.2014.07.003

Seminars in Arthritis and Rheumatism

Volume 44, Issue 3, December 2014, Pages 371-379

https://doi.org/10.1016/j.semarthrit.2014.07.003 Get rights and content

Abstract

Objective

Osseous sarcoidosis has been infrequently reported. We aimed to characterize the distribution of lesions, clinical presentation, treatment, and outcomes for osseous sarcoidosis.

Methods

Cases of osseous sarcoidosis were identified by directed inquiry to clinicians and electronic query. Cases were defined as having pathologic evidence of non-caseating granulomas on bone biopsy or evidence of osseous lesions on imaging attributable to sarcoidosis in patients with known sarcoidosis. Detailed characteristics were obtained by medical record review.

Results

We identified a total of 20 cases of osseous sarcoidosis. Osseous lesions were detected by imaging during the initial sarcoidosis presentation in 60% of cases. In those who had a prior diagnosis of sarcoidosis, the median duration of sarcoidosis before detection of osseous involvement was 4.3 years. Symptoms were present in 50% of cases. All cases had more than one bone involved. The axial skeleton was involved in the majority of cases (90%), primarily the pelvis and the lumbar spine. Most cases required no treatment (55%); a minority of cases (45%) were treated, most often with prednisone, methotrexate, or hydroxychloroquine. Two cases required multiple immunosuppressants, including tumor necrosis factor inhibitors, for refractory symptomatic osseous sarcoidosis. Treated cases were younger than those who were untreated. At last follow-up, most cases (85%) were asymptomatic from osseous lesions.

Conclusions

In this case series of osseous sarcoidosis from a single center, most patients had multiple bones affected and had other systemic manifestations of sarcoidosis. A minority required treatment for relief of symptoms, and most cases were asymptomatic at last follow-up.

Introduction

Sarcoidosis is a systemic inflammatory disease characterized by non-caseating granulomas in affected organs [1], [2]. Sarcoidosis can affect any organ system in the body. Pulmonary involvement is most common, affecting more than 90% of patients with this diagnosis [3]. Typical chest radiographic findings include hilar and mediastinal lymphadenopathy with or without pulmonary parenchymal opacities [3]. Musculoskeletal manifestations of sarcoidosis are less common, affecting about 10–25% of patients [3]. Löfgren׳s syndrome, comprised of hilar lymphadenopathy, erythema nodosum, and ankle periarthritis, is a well-recognized musculoskeletal presentation of sarcoidosis [4], [5]. Other musculoskeletal manifestations of sarcoidosis are only sporadically described in the literature.

Previously, a large study of sarcoidosis reported only four patients with bone or joint involvement out of 736 sarcoidosis patients (0.5%) [3]. The frequency of osseous sarcoidosis in other studies ranges from 3% to 13% [6]. However, bone involvement may be more common than previously reported, since only about half of osseous sarcoidosis patients are symptomatic and many do not obtain advanced imaging [7]. Some have noted that bone involvement in sarcoidosis occurs very rarely in the absence of skin lesions [8]. A study that used magnetic resonance imaging to evaluate sarcoidosis patients with musculoskeletal symptoms and normal plain radiographs found occult bone lesions that resembled metastases and were predominant in the axial skeleton [9].

Prior reports characterized osseous sarcoidosis as cystic changes in the hands and feet in patients with soft tissue swelling or facial bone involvement in patients with lupus pernio [10]. Other areas of involvement are rarely described in prior literature. Radiographically, bone lesions have been described as permeative, destructive, or lytic [11]. Imaging may not reliably differentiate osseous sarcoidosis from metastatic cancer, particularly in the spine and the pelvis, prompting the need for bone biopsy [12]. Treatment and outcomes of patients with osseous sarcoidosis have been infrequently described. We aimed to describe the distribution of bone lesions, clinical presentation, treatment strategies, and outcomes of patients with osseous sarcoidosis at our academic center.

Section snippets

Case ascertainment

Potential cases of osseous sarcoidosis at Brigham and Women׳s Hospital (BWH), an academic hospital in Boston, Massachusetts, were identified by directed inquiry to rheumatologists in the Division of Rheumatology, Immunology, and Allergy and pulmonologists in the Division of Pulmonary and Critical Care Medicine. Electronic health record queries were also performed to identify potential cases and to estimate the prevalence of osseous sarcoidosis among all sarcoidosis patients at BWH during the

Results

A total of 20 cases of osseous sarcoidosis seen at BWH between 1994 and 2013 were identified. During this period, we identified a total of 1316 patients with probable sarcoidosis by electronic health record query at BWH. The prevalence of osseous sarcoidosis among all sarcoidosis patients was therefore estimated to be 1.5%. Overall, 10 cases had bone biopsies with pathologic evidence of non-caseating granulomas. There was evidence of osseous involvement by imaging studies and clinical

Discussion

We described the clinical presentation, treatment, and outcomes of 20 patients with osseous sarcoidosis from an academic center. In our study, all patients with osseous sarcoidosis had more than one bone involved, and the lumbar spine and pelvis were the most commonly affected bones. Half of the cases were symptomatic from osseous lesions. Symptoms included back, thigh, or pelvic pain as well as localized edema, erythema, and tenderness to palpation in the hands and feet. Several patients were

Conclusion

We described 20 patients with osseous sarcoidosis seen at our institution. In contrast to previous reports, we found that spinal and pelvic lesions were common among sarcoidosis patients with bone involvement. All but one of the patients in our study had other typical systemic features of sarcoidosis, and all patients with osseous sarcoidosis had more than one bone affected. A minority of patients required treatment of their osseous sarcoidosis for relief of symptoms, and most patients were

Acknowledgments

The authors would like to acknowledge Ronald Anderson, MD, Rebecca Baron, MD, Paul Dellaripa, MD, Tracy Doyle, MD, William Docken, MD, Hilary Goldberg, MD, Matthew Liang, MD, MPH, Elinor Mody, MD, Danielle Morse, MD, and Simon Helfgott, MD, for their help in identifying cases for this study. We thank Matt Hunninghake, MD, for additional guidance. We would also like to thank all of the patients and staff at Brigham and Women׳s Hospital.

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Osseous sarcoidosis: Clinical characteristics, treatment, and outcomes—Experience from a large, academic hospital

Abstract

Objective

Methods

Results

Conclusions

Introduction

Section snippets

Case ascertainment

Results

Discussion

Conclusion

Acknowledgments

Am J Med

Am J Med

Rheum Dis Clin North Am

Semin Oncol

Radiol Clin North Am

Sarcoidosis

J Am Med Assoc

Clinical characteristics of patients in a case control study of sarcoidosis

Am J Respir Crit Care Med

Osteoarticular involvement in a series of 100 patients with sarcoidosis referred to rheumatology departments

J Rheumatol

A worldwide review of sarcoidosis

Ann N Y Acad Sci

Sarcoidosis of bone

Q J Med

Dermatological aspects of sarcoidosis

Q J Med

MRI of sarcoidosis patients with musculoskeletal symptoms

Am J Roentgenol

Bone sarcoidosis

Curr Opin Rheumatol

Can sarcoidosis and metastatic bone lesions be reliably differentiated on routine MRI?

Am J Roentgenol