Rheumatology Advance Access originally published online on September 23, 2008
Rheumatology 2008 47(11):1733-1734; doi:10.1093/rheumatology/ken366
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Ankylosing spondylitis and pulmonary sarcoidosis—a case report and discussion of the literature
1Department of Rheumatology, St Georges Hospital, Tooting, London, UK
Correspondence to: S. Levy, Department of Rheumatology, Kings College Hospital, Denmark Hill, London SE5 9RS, UK. E-mail: sarahlevy{at}doctors.net.uk
SIR, Co-occurrence of sacroiliitis and sarcoidosis is rarely reported, but when it occurs it is a cause of diagnostic confusion [1]. The two conditions may co-exist either due to sarcoid deposition within the SI joint or due to the development of two distinct conditions (sarcoid and SpA) within the same patient [1]. Recent reports of the development of pulmonary sarcoidosis in patients with AS treated with anti-TNF-
therapy makes correct diagnosis in these cases critical [2, 3]. We present the case of a 31-yr-old man with a 23-yr history of AS, who was found to have radiological and histological evidence of pulmonary sarcoidosis while undergoing preliminary assessment for anti-TNF- therapy.
The patient came under the care of our clinic in 1999. Plain radiographs at that time showed sclerosis and ankylosis of both SI joints (Fig. 1a), and Schober's test and chest expansion were well maintained at 5 cm. Auscultation of the chest was normal. Initially he was managed with oral naproxen and physiotherapy. Oral SSZ was started when his symptoms worsened.
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In 2005, he reported increasing spinal pain and stiffness and examination revealed that his spinal movements had deteriorated, with Schober's test and chest expansion at 2 cm. At this point, the use of TNF-
blocking therapy was discussed with the patient. A routine chest radiograph at this time, however, revealed reticular–nodular shadowing mainly in the mid- and upper zones (Fig. 1b). High-resolution CT scanning of the lungs showed enlarged mediastinal and hilar lymph nodes with extensive mid- and upper zone fibrosis consistent with pulmonary sarcoidosis. Transbronchial lung biopsy revealed non-necrotizing granulomata and bronchioalveolar lavage revealed a chronic inflammatory picture. There was no evidence of tuberculosis on prolonged culture. Pulmonary function tests showed an FEV1 of 2.73 l (69% of predicted), an FVC of 3.0 l (62% of predicted) and a transfer factor of 1.68 (107% of predicted). There were no previous chest radiographs within the hospital for comparison. Direct questioning revealed that the patient had had symptoms of breathlessness since adolescence, but had not reported this and had modified his activities to avoid exertion. Later in life he had found that his spinal symptoms were the main limit to his activity. Tissue typing revealed that along with HLA B27 the patient carried DR4, which has been associated with late onset of sarcoidosis, and DQB1*06, which has been associated with susceptibility to sarcoid [4].
There have been only five other reported cases of sarcoidosis developing in a patient with established AS [1, 5–7]. All of these patients were HLA-B27 positive and presented initially with sacroiliitis but then went on to develop pulmonary sarcoidosis (in one case with skin nodules [1]) with non-necrotizing granulomata on lung biopsy. There have been nine reported cases (six before 1975) in which features of SpA and sarcoidosis have co-existed [1, 5, 6, 8]. In one case, biopsy of the SI joint revealed sarcoid, and in the four cases where HLA typing was available, all were B27 negative but carried HLA types that have been associated with sarcoidosis (B8, B13, B35 and A9), making sarcoid of the SI joint the most likely diagnosis.
A recent cross-sectional study of a UK sarcoid population found that radiological sacroiliitis occurred in 6.6% of the patients compared with 1.9% of the normal population [9]. These data, however, do not support a true association between SpA and sarcoidosis, as only one of the four patients found to have sacroiliitis on X-ray was HLA-B27 positive. It may be, however, that sarcoid of the SI joint may be more common than previously thought. A single European study found that isolated pulmonary sarcoid was associated with HLA-B27 [10], making a true association between SpA and sarcoid possible, but this finding has not been replicated and the relatively high prevalence of the two conditions (1:1000 for AS and 0.04–64/1000 for sarcoidosis [7]) makes sporadic co-occurrence likely.
In conclusion, co-existence of SpA and pulmonary sarcoid is rare, but sarcoid of the SI joint may be more common than previously thought. Treatment with TNF- blockade may precipitate de novo pulmonary sarcoid and with the increasing use of these drugs for the treatment of SpA accurate diagnosis in patients who present with both sacroiliitis and sarcoid is critical. The clinician should be aware that patients with SpA of sufficient severity to warrant treatment with anti-TNF agents may not report symptoms of pulmonary sarcoid due to limitation of their activity by musculoskeletal symptoms. We would therefore recommend that all AS patients undergo thorough assessment and monitoring of respiratory function prior to and during treatment with TNF- blockers.
Disclosure statement: The authors have declared no conflicts of interest.
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