Rheumatologic manifestations of sarcoidosis

Abstract

Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration-approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients' symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis.

Figure 1

photograph of the frontal area showing erythematous rash. Biopsy confirmed features of discoid lupus and noncaseating granulomas in the same lesion.

Figure 2

resence of noncaseating granulomas in muscle in sarcoid myopathy.

Figure 3

iagnosis and management of patients with sarcoid arthritis. MTX, methotrexate; SZA, sulfasalazine; DMARD, disease modifying antirheumatic drugs (hydroxychloroquine, MTX, SZA, minocycline, azathioprine, leflunomide); NSAIDs, nonsteroidal antiinflammatory drugs; PT/OT, physical therapy/occupational therapy; biologics, anti-TNF agents (Infliximab, adalimumab), co-stimulatory agents (abatacept); B-cell depleting agents (rituximab); combination therapy, multiple DMARDs and/or a DMARD plus a biologic. *Low-dose steroids, <10 to 20 mg prednisone daily **Suboptimal response to MTX, intolerance to drug, lack of satisfactory efficacy on dosage up to 25 mg/week, or a contraindication to medication use. ***DMARD failure, progressive disease or drug intolerance. ****Methylprednisone preferred over prednisone if prednisone has been used prior.

Figure 4

and photograph shows (A) proximal interpharyngeal (PIP) joint swelling and erythema, (B) distal interpharyngeal (DIP) joint swelling and erythema, and (C) dystrophic nail changes, without evidence of pitting.

Figure 5

and x-ray from patient in Figure 4 reveals osseous erosions bilaterally. Big circle: Lacy reticular pattern characteristic of sarcoidosis—fifth digit and soft tissue swelling. Small circles: Acroosteolysis of distal phalanx tufts. Long arrow: Punched-out granuloma neck proximal phalanx of ring finger. Short arrows: Granulomatous erosion.

Figure 6

pine magnetic resonance imaging without contrast STIR (short tau inversion recovery), sagittal view demonstrating axial sarcoid disease. Circle indicates pathological L4 compression fracture. Arrows indicate multiple sarcoid bony lesions.