Arthritis mutilans is a rare medical condition involving severe inflammation damaging the joints of the hands and feet, and resulting in deformation and problems with moving the affected areas; it can also affect the spine. As an uncommon arthropathy, arthritis mutilans was originally described as affecting the hands, feet, fingers, and/or toes, but can refer in general to severe derangement of any joint damaged by arthropathy. First described in modern medical literature by Marie and Leri in 1913, in the hands, arthritis mutilans is also known as opera glass hand (la main en lorgnette in French), or chronic absorptive arthritis. Sometimes there is foot involvement in which toes shorten and on which painful calluses develop in a condition known as opera glass foot, or pied en lorgnette.
Signs and symptoms
For a person with arthritis mutilans in the hands, the fingers become shortened by arthritis, and the shortening may become severe enough that the hand looks paw-like, with the first deformity occurring at the interphalangeal and metacarpophalangeal joints. The excess skin from the shortening of the phalanx bones becomes folded transversely, as if retracted into one another like opera glasses, hence the description la main en lorgnette. As the condition worsens, luxation, phalangeal and metacarpal bone absorption, and skeletal architecture loss in the fingers occurs.
Arthritis mutilans occurs mainly in people who have pre-existing psoriatic arthritis, but can occur, if less often, in advanced rheumatoid arthritis; it can also occur independently. Psoriasis and psoriatic arthritis are interrelated heritable diseases, occurring with greater heritable frequency than rheumatoid arthritis, primary Sjogren's syndrome and thyroid disease. Psoriasis affects 2–3% of the Caucasian population, and psoriatic arthritis affects up to 30% of those. Arthritis mutilans presents in about 5–16% of psoriatic arthritis cases, involves osteolysis of the DIP and PIP joints, and can include bone edema, bone erosions, and new bone growth. Most often psoratic arthitis is seronegative for rheumatoid factor (occurring in only about 13% of cases), and has genetic risk factor overlap with ankylosing spondylitis with HLA-B27, IL-23R77, and IL-1, however, as of 2016, immunopathogenesis is unclear.
Enthesitis can assist in differentiating arthritis mutilans' parent condition psoriatic arthritis from rheumatoid arthritis and osteoarthritis, with evidence in plain radiographs (x-rays) and MRI as periostitis, new bone formation, and bone erosions. Dactylitis, spondylitis and sacroiliitis are common with the parent condition psoriatic arthritis, but are not in rheumatoid arthritis. MRI bone edema scores are high in arthritis mutilans and correlate with radiographic measures of joint damage, although they may not correlate with disease activity. A source of significant pain, bone marrow edema (or lesions, using newer terminology), can be detected on MRI or with ultrasonography by signals of excessive water in bone marrow. Specifically, bone marrow edema can be detected within bone on T1-weighted images as poorly defined areas of low signal, with a high signal on T2-weighted fat-suppressed images. Comparatively, with arthritis mutilans in rheumatoid arthritis, bone marrow edema often involves the subchondral bone layer, while the condition as a subtype of psoriatic arthritis includes a greater extent of marrow edema, expanding to diaphysis.
The bone edema in arthitis mutilans can be treated with TNF inhibitors in the short term: a 2007 study found that the bone edema associated with psoriatic arthritis (of which arthitis mutilans is a subtype) responded to TNF inhibitors with "dramatic" improvement, but the study was not determinative of whether TNF inhibitors would prevent new bone formation, bone fusion, or osteolysis (bone resorption).
Although a 2011 research article stated that disagreements between hand surgeons and rheumatologists remain regarding the indications, timing and effectiveness of rheumatoid hand surgery, arthritis mutilans may be successfully treated by iliac-bone graft and arthrodesis of the interphalangeal joints and the metacarpophalangeal joint in each finger.
Arthritis mutilans' parent condition psoriatic arthritis leaves people with a mortality risk 60% higher than the general population, with premature death causes mirroring those of the general population, cardiovascular issues being most common. Life expectancy for people with psoriatic arthritis is estimated to be reduced by approximately 3 years.
- NIH Genetics Home Reference, Psoriatic arthritis. Accessed 2016-11-06.
- Nielsen, Børge, and Egill Snorrason, Arthritis mutilans, Acta Radiologica [Old Series], November 1946 os-27: 607-616, doi:10.1177/028418514602700605
- Nelson, L. S., The Opera-Glass Hand in Chronic Arthritis. "La Main en Lorgnette" of Marie and Leri, Journal of Bone and Joint Surgery, 20:1045-1049, 1938-10-1. Accessed 2016-11-6. Archived version at archive.org.
- Solomon, W.M., Stecher, R.M., Chronic Absorptive Arthritis or Opera-Glass Hand: report of 8 cases, Annals of the Rheumatic Diseases, September 1950, 9(3):209–220, PMID 14783359. PMC 1030771.
- Froimson, Avrum I., M.D., Hand Reconstruction in Arthritis Mutilans A Case Report, Journal of Bone and Joint Surgery, 53-A:7, 1971-10-1, pp. 1377–1382 PMID 4939959.
- Eisenstadt, H. B., and Eggers, G.W.N., Arthritis Mutilans, Journal of Bone and Joint Surgery, 37-A:337-346, April 1955. PMID 14367425. Accessed 2016-11-6.
- Nograles, K., Brasington, R. and Bowcock, A., New insights into the pathogenesis and genetics of psoriatic arthritis, Nature clinical practice. Rheumatology, 5(2):83–91, Feb. 2009. PMC 2790861, doi:10.1038/ncprheum0987
- Sudoł-Szopińska I., Matuszewska G., Kwiatkowska B., Pracoń G., Diagnostic imaging of psoriatic arthritis. Part I: etiopathogenesis, classifications and radiographic features, Journal of Ultrasonography, 16(64):65-77, March 2016. doi:10.15557/JoU.2016.0007 PMC 4834372
- Rodgers M., Epstein D., Bojke L., et al, Etanercept, Infliximab and Adalimumab for the Treatment of Psoriatic Arthritis: A Systematic Review and Economic Evaluation, Health Technology Assessment, No. 15.10, Southampton (UK): NIHR Journals Library, Feb. 2011, p.1. Accessed 2016-11-06.
- Tan Y. M., et al, MRI bone oedema scores are higher in the arthritis mutilans form of psoriatic arthritis and correlate with high radiographic scores for joint damage, Arthritis Research & Therapy, 11(1):R2, Jan. 6, 2009. PMID 19126234. doi:10.1186/ar2586. Report of a study investigating MRI features of bone disease in arthritis mutilans as a form of psoriatic arthritis.
- Eriksen, E., Treatment of bone marrow lesions (bone marrow edema), BoneKEy Reports 4, Article 755, Nov. 25, 2015, Journal of the International Bone and Mineral Society. doi:10.1038/bonekey.2015.124.
- McQueen F.M., Benton N., Perry D., Crabbe J., et al, Bone edema scored on magnetic resonance imaging scans of the dominant carpus at presentation predicts radiographic joint damage of the hands and feet six years later in patients with rheumatoid arthritis, Arthritis and Rheumatology, 48:7, pp. 1814–1827, July 2003. doi:10.1002/art.11162.
- Sudoł-Szopińska I. and Pracoń G., Diagnostic imaging of psoriatic arthritis. Part II: magnetic resonance imaging and ultrasonography, Journal of Ultrasonography, 16(65):163–174, June 2016. doi:10.15557/JoU.2016.0018 PMC 4954862
- Marzo-Ortega H., McGonagle D., et al, Efficacy of infliximab on MRI‐determined bone oedema in psoriatic arthritis], Annals of Rheumatic Diseases, 66(6):778–781, Jun 2007. PMID 17185324, doi:10.1136/ard.2006.063818, PMC 1954680.
- Chung, Kevin C., and Pushman, Allison G., Current Concepts in the Management of the Rheumatoid Hand, Journal of Hand Surgery, April 2011, 36(4):736–747. PMID 21463736. doi:10.1016/j.jhsa.2011.01.019. Accessed 2014-4-11.
- Nalebuff EA, Garrett J (1976). "Opera-glass hand in rheumatoid arthritis". J Hand Surg Am. 1 (3): 210–20. doi:10.1016/s0363-5023(76)80040-8. PMID 1018089.
- Gladman DD, Antoni C, Mease P, Clegg DO, Nash P., Psoriatic arthritis: epidemiology, clinical features, course, and outcome], Annals of the Rheumatic Diseases, 64(Suppl 2):ii14–ii17, Mar. 2005. doi:10.1136/ard.2004.032482, PMC 1766874.