Rheumatology Advance Access originally published online on July 8, 2008
Rheumatology 2008 47(9):1433-1435; doi:10.1093/rheumatology/ken252
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Sarcoidosis and inclusion body myositis
1Department of Neurological Sciences and Vision, Section of Clinical Neurology, University of Verona, Verona, 2Unità Operativa di Medicina, 3Unità Operativa di Neurologia, Hospital of Thiene, Vicenza and 4Neurological Clinic, Section for Neuromuscular Diseases and Neuropathies, University Hospital ‘Spedali Civili’ of Brescia, Brescia, Italy
Correspondence to: G. Tomelleri, Department of Neurological Sciences and Vision, Section of Clinical Neurology, Policlinico G.B. Rossi, P.le L.A. Scuro 10, 37134 Verona, Italy. E-mail: giuliano.tomelleri{at}univr.it
SIR, Sarcoidosis, a multisystem disease of unknown aetiology, is pathologically characterized by the presence of non-caseating granuloma in the affected organs [1,2]. Muscle involvement is quite frequent; however the association of a sarcoid myopathy with IBM, a chronic inflammatory myopathy of the adults, has been reported only in six cases [1–5]. We report two more patients with muscle biopsies harbouring the pathological hallmarks of both sarcoidosis and IBM.
A 54-yr-old-man had 1-yr history of myalgia on climbing stairs and difficulty in lifting heavy weights. On examination he had proximal muscle weakness at four limbs and atrophy of the third distal segment of thighs and brachial biceps. Tendon reflexes were lost and sensation was normal. Blood test results were normal and serum Creatine Kinase (CK) was 2-fold the normal value. Chest CT showed bilateral hilar lymphoadenopathy without infiltration (Stage 1). Electromyography (EMG) recorded myopathic and neurogenic changes. Biopsy of brachial biceps muscle showed numerous non-caseating granulomas in the connective tissue and within the muscle bundles (Fig. 1A); no cellular necrosis was observed. Muscle fibre membranes did not express MHC class I (MHCI) and no amyloid deposits were detected within the myofibres. A diagnosis of sarcoid myopathy with pulmonary involvement was made. The patient was treated with prednisone for 2 yrs without any improvement. At the age of 58 yrs, he noted progressive left foot drop that forced him to use the handrail to climb stairs; he rose from the squatting position with some difficulty and was able to do only three knee bends consecutively. Arm movements were full and muscle strength was unchanged. Blood tests, chest CT and EMG were unchanged. A second biopsy from vastus lateralis muscle showed numerous non-caseating granulomas and the typical features of IBM, i.e. rimmed vacuoles, eosinophilic inclusions and non-necrotic fibres invaded by inflammatory cells (Fig. 1B), amyloid deposits and amyloid-β-immunoreactive inclusions (Fig. 1E and F); the sarcolemma of many fibres expressed MHCI.
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The second patient, a 48-yr-old man, had a diagnosis of lung sarcoidosis on the basis of bilateral hilar lymphadenopathy with infiltration by chest radiographs (Stage 2) supported by the histological evidence of non-caseating granulomas by transbronchial biopsy. He did not complain of any muscular symptoms and the neurological examination was normal. The patient responded to treatment with prednisone. At the age of 55 yrs, he developed dysaesthesia followed by progressive muscle weakness. On examination, 5 yrs later, proximal and distal muscles of the four limbs were weak, sensation was reduced distally and tendon reflexes were lost. EMG was consistent with a sensory-motor polyneuropathy. A sural nerve biopsy showed severe loss of myelinated fibres without inflammatory infiltrates or amyloid deposits. Muscle weakness worsened in time. When the patient was 62-yr old, CK was 8-fold the normal value. Biopsy of vastus lateralis muscle showed fibre size variability, small groups of atrophic fibres, few necrotic fibres and endomisial fibrosis. A small non-caseating granuloma and rimmed vacuoles inside many fibres with few eosinophilic inclusions were present (Fig. 1C and D). A few muscle cells expressed MHCI on sarcolemma.
The association of IBM and muscular sarcoidosis is probably casual, nevertheless, our observation, together with previous reports [3–5], raises the likelihood of a link between these two apparently unrelated diseases. From 2952 consecutive muscle biopsies performed in our department, we identified six patients with pulmonary sarcoidosis (0.20%) and 27 patients with IBM who represent 0.91% of our patient population, a frequency that ranges between those reported by other authors (0.4–1.3%) [6]. Among the six patients with pulmonary sarcoidosis, two had muscular sarcoidosis and IBM, two patients had a non-specific myopathy and two a normal muscle. In addition, 7.4% of our IBM patients had sarcoidosis; this is a considerable frequency compared with previous papers reporting the association of IBM with other autoimmune disorders in 1–4% of the cases [7,8]. It is relevant to underline that IBM and sarcoidosis are mediated by Th1-driven immune reactions [9]. Interestingly, in our first patient the histological features of both sarcoidosis and IBM were clearly demonstrated only at the second muscle biopsy thus suggesting that sarcoid myopathy may have promoted IBM. Also the clinical course supports this hypothesis since the asymmetrical muscle weakness that can be observed in IBM is not usually reported in chronic sarcoid myopathy. Even though we cannot exclude that the discrepancy observed between the first and the second muscle biopsy may be due to the different muscles chosen for the pathological examination, we consider it unlikely since IBM features are usually present at the four limbs [10]. Another possibility is that the presentation of the two diseases occurs at different times, as it frequently happens in autoimmune disorders; alternatively IBM could represent a common phenotypic end-point of muscular sarcoidosis [11].
We emphasize the importance of a careful clinical follow-up, eventually with a second muscle biopsy, in patients who present an unusual course of muscular sarcoidosis or an uncommon pattern of muscle weakness.
Disclosure statement: The authors have declared no conflicts of interest.
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