Pigmented villonodular synovitis of the hip in systemic lupus erythematosus: A case report

Department of Nephrology, Medizinische Poliklinik, University of Munich, Munich, Germany. .
Journal of Medical Case Reports 09/2011; 5:443. DOI: 10.1186/1752-1947-5-443
Source: PubMed


Pigmented villonodular synovitis is a rare disease of unknown etiology mostly affecting the knee and foot. Until now an association with autoimmune diseases has not been reported.
The diagnosis of systemic lupus erythematosus was made in a 15-year-old Caucasian girl based on otherwise unexplained fatigue, arthralgia, tenosynovitis, leukopenia, low platelets and the presence of antinuclear and deoxyribonucleic antibodies. At the age of 20 a renal biopsy revealed lupus nephritis class IV and she went into complete remission with mycophenolate mofetil and steroids. She was kept on mycophenolate mofetil for maintenance therapy. At the age of 24 she experienced a flare-up of lupus nephritis with nephrotic syndrome and new onset of pain in her right hip. Magnetic resonance imaging, arthroscopy and subtotal synovectomy identified pigmented villonodular synovitis as the underlying diagnosis. Although her systemic lupus erythematosus went into remission with another course of steroids and higher doses of mycophenolate mofetil, the pigmented villonodular synovitis persisted and she had to undergo open synovectomy to control her symptoms.
Systemic lupus erythematosus is associated with many different musculoskeletal manifestations including synovitis and arthritis. Pigmented villonodular synovitis has not previously been reported in association with systemic lupus erythematosus, but as its etiology is still unknown, the present case raises the question about a causal relationship between systemic lupus erythematosus and pigmented villonodular synovitis.

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    ABSTRACT: Pigmented villonodular synovitis (PVNS) is a rare condition, most commonly involving the knee joint. PVNS is locally aggressive and can invade and destroy surrounding soft tissue and bone, leading to anatomical and functional deterioration of the affected joint. Localized PVNS is an unusual presentation of the disease, generally consisting of a nodular lesion protruding into the articular cavity. Localized PVNS of the knee can mimic other joint disorders which may pose a challenge for a correct diagnosis. Given the locally aggressive behavior of PVNS, prompt identification and excision of the lesion are instrumental to avoid complications. Here, we report a rare case of localized cystic PVNS involving the anterior cruciate ligament of the knee in a 32-year-old woman with persistent knee pain, in whom magnetic resonance imaging was inconclusive. The diagnosis was achieved via arthroscopy and histology. We also present a concise review of the literature on this pathological entity as well as a discussion on the differential diagnosis between localized PVNS and other intra-articular cystic lesions.
    International journal of immunopathology and pharmacology 10/2012; 25(4):1131-6. · 1.62 Impact Factor
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    ABSTRACT: A 56-year-old man developed pigmented villonodular synovitis (PVNS) 3 years after he was diagnosed with rheumatoid arthritis (RA). He had been successfully treated with methotrexate, leflunomide, sulfadiazine, and intra-articular knee injection of etanercept (tumor necrosis factor α inhibitor) in 2010. He stopped all drugs for arthritis 1 year later for disease remission. He was readmitted for right knee pain and swelling in 2013, when the magnetic resonance imaging and arthroscopy of the right knee indicated PVNS. Following surgical resection, the patient was doing well after 1 year. This rare case is the first reported case in English-language literature of PVNS of the knee seen in RA patients and illustrates the importance of differential diagnosis of this condition with synovial cysts, which are commonly found in RA.
    Journal of clinical rheumatology: practical reports on rheumatic & musculoskeletal diseases 07/2014; 20(5). DOI:10.1097/RHU.0000000000000119 · 1.08 Impact Factor

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