Vertebral Sarcoidosis: A Rare but Important Manifestation of the Disease to Promptly Diagnose and Treat

Abstract

INTRODUCTION: Sarcoidosis is a systemic disease defined by noncaseating granulomatous inflammation of uncertain etiology. It can affect most organ systems with lung involvement occurring in about 90 percent of cases. Skeletal involvement is much less common with a prevalence of 1 to 13 percent. Vertebral involvement is very rare with a variable disease course and no clear treatment guidelines. We describe a patient with progressive lung involvement and vertebral lesions. CASE PRESENTATION: Our patient is a 55 year old female with presumptive sarcoidosis based upon a skin biopsy. She was referred to our clinic for progressive parenchymal nodules and mediastinal lymphadenopathy. She reported a nonproductive cough as well as right hip and shoulder pain. Her exam was notable only for pain with active range of motion of the right hip and shoulder. A complete blood count, chemistry, angiotensin converting enzyme, and inflammatory markers were normal. Spirometry revealed a mild restrictive pattern and a normal DLCO. A cardiac PET was performed to evaluate for heart involvement and incidentally noted avid vertebral lesions. A whole body PET confirmed the presence of avid vertebral lesions as well as significant hypermetabolic uptake in the right acetabulum, femur, left ischium, and bilateral iliac crests. She received a 3 month prednisone taper and a repeat PET revealed progression of her vertebral and parenchymal lesions. Bronchoscopy with transbronchial needle aspirations of her mediastinal lymph nodes and endobronchial biopses revealed granulomatous inflammation, and broncho-alveolar lavage was unremarkable. Additionally, a CT guided needle biopsy of her iliac crest was pursued, and ultimately revealed noncaseating granulomatous inflammation as well. DISCUSSION: Vertebral sarcoidosis is a very rare condition with only a few reported cases in the medical literature. Patients may present with back pain, radicular symptoms, or may be asymptomatic. The lesions may be sclerotic or lytic, and potential complications include vertebral instability with spinal cord compression. It is imperative to establish a tissue diagnosis to ensure that metastatic tumors, fungal infections, and Pott's disease, among others are not present. Given the paucity of cases, therapeutic regimens remain anectodal. Variable success with prednisone and methotrexate have been reported, with more recent reports demonstrating a role for infliximab in refractory cases. Given our patient's symptoms, a longer trial of high dose prednisone will be attempted followed by infliximab if improvement is not observed on repeat imaging. CONCLUSIONS: Vertebral sarcoidosis is a rare manifestation of this disease but one that needs to be considered in the appropriate clinical setting, as its progression may be devastating if unrecognized.