Arthritis mutilans

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.[1] 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.[2] 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.[3][4] 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.[5][6]

Arthritis mutilans
SpecialtyRheumatology 

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.[5]

Cause

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.[5][6] Psoriasis and psoriatic arthritis are interrelated heritable diseases, occurring with greater heritable frequency than rheumatoid arthritis, primary Sjögren syndrome and thyroid disease.[7] Psoriasis affects 2–3% of the Caucasian population, and psoriatic arthritis affects up to 30% of those.[7] 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.[8] Most often psoriatic arthritis is seronegative for rheumatoid factor (occurring in only about 13% of cases[9]), and has genetic risk factor overlap with ankylosing spondylitis with HLA-B27, IL-23R77, and IL-1,[7] however, as of 2016, immunopathogenesis is unclear.[8]

Diagnosis

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.[7] Dactylitis, spondylitis and sacroiliitis are common with the parent condition psoriatic arthritis, but are not in rheumatoid arthritis.[9] 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.[10] 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.[11] 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.[12] 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.[10][13]

Treatment

Medication

The bone edema in arthritis 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 arthritis 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).[10][14]

Surgical

Although a 2011 research article stated that disagreements between hand surgeons and rheumatologists remain regarding the indications, timing and effectiveness of rheumatoid hand surgery,[15] arthritis mutilans may be successfully treated by iliac-bone graft and arthrodesis of the interphalangeal joints and the metacarpophalangeal joint in each finger.[5][16]

Outcomes

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.[9][17] Life expectancy for people with psoriatic arthritis is estimated to be reduced by approximately 3 years.[9]

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References
  1. National Institutes of Health Genetics Home Reference, Psoriatic arthritis. Accessed 2016-11-06.
  2. Nielsen Børge, Snorrason Egill (1946). "Arthritis mutilans". Acta Radiologica. 27: 607–616. doi:10.1177/028418514602700605.
  3. Nelson, L. S., The Opera-Glass Hand in Chronic Arthritis. "La Main en Lorgnette" of Marie and Leri Archived 2016-11-07 at the Wayback Machine, Journal of Bone and Joint Surgery, 20:1045-1049, 1938-10-1. Accessed 2016-11-6. Archived version at archive.org.
  4. Solomon WM, Stecher RM (September 1950). "Chronic absorptive arthritis or opera-glass hand: report of 8 cases". Ann. Rheum. Dis. 9 (3): 209–20. doi:10.1136/ard.9.3.209. PMC 1030771. PMID 14783359.
  5. Froimson AI (October 1971). "Hand reconstruction in arthritis mutilans. A case report". J Bone Joint Surg Am. 53 (7): 1377–82. doi:10.2106/00004623-197153070-00014. PMID 4939959.
  6. Eisenstadt HB, Eggers GW (April 1955). "Arthritis mutilans (doigt, main, pied enlorgnette)". J Bone Joint Surg Am. 37-A (2): 337–46. doi:10.2106/00004623-195537020-00012. PMID 14367425.
  7. Nograles K., Brasington R., Bowcock A. (2009). "New insights into the pathogenesis and genetics of psoriatic arthritis". Nature Clinical Practice. Rheumatology. 5 (2): 83–91. doi:10.1038/ncprheum0987. PMC 2790861. PMID 19182814.CS1 maint: multiple names: authors list (link)
  8. Sudoł-Szopińska I., Matuszewska G., Kwiatkowska B., Pracoń G. (2016). "Diagnostic imaging of psoriatic arthritis. Part I: etiopathogenesis, classifications and radiographic features". Journal of Ultrasonography. 16 (64): 65–77. doi:10.15557/JoU.2016.0007. PMC 4834372. PMID 27104004.CS1 maint: multiple names: authors list (link)
  9. Rodgers M., Epstein D., Bojke L.; et al. (2011). "Etanercept, Infliximab and Adalimumab for the Treatment of Psoriatic Arthritis: A Systematic Review and Economic Evaluation". Health Technology Assessment. 15 (10): 1. doi:10.3310/hta15100. PMID 21333232.CS1 maint: multiple names: authors list (link)
  10. Tan YM, Østergaard M, Doyle A, Dalbeth N, Lobo M, Reeves Q, Robinson E, Taylor WJ, Jones PB, Pui K, Lee J, McQueen FM (2009). "MRI bone oedema scores are higher in the arthritis mutilans form of psoriatic arthritis and correlate with high radiographic scores for joint damage". Arthritis Res. Ther. 11 (1): R2. doi:10.1186/ar2586. PMC 2688232. PMID 19126234.CS1 maint: multiple names: authors list (link). Report of a study investigating MRI features of bone disease in arthritis mutilans as a form of psoriatic arthritis.
  11. Eriksen E (2015). "Treatment of bone marrow lesions (bone marrow edema)". BoneKEy Reports. 4: 755. doi:10.1038/bonekey.2015.124. PMC 4662576. PMID 26644910.
  12. McQueen F.M., Benton N., Perry D., Crabbe J.; et al. (2003). "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): 1814–1827. doi:10.1002/art.11162. PMID 12847674.CS1 maint: multiple names: authors list (link)
  13. Sudoł-Szopińska I., Pracoń G. (2016). "Diagnostic imaging of psoriatic arthritis. Part II: magnetic resonance imaging and ultrasonography". Journal of Ultrasonography. 16 (65): 163–174. doi:10.15557/JoU.2016.0018. PMID 27446601.
  14. Marzo-Ortega H., McGonagle D.; et al. (2007). "Efficacy of infliximab on MRI‐determined bone oedema in psoriatic arthritis". Annals of the Rheumatic Diseases. 66 (6): 778–781. doi:10.1136/ard.2006.063818. PMC 1954680. PMID 17185324.
  15. Chung Kevin C., Pushman Allison G. (2011). "Current Concepts in the Management of the Rheumatoid Hand". Journal of Hand Surgery. 36 (4): 736–747. doi:10.1016/j.jhsa.2011.01.019. PMC 3086569. PMID 21463736.
  16. 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.
  17. Gladman DD, Antoni C, Mease P, Clegg DO, Nash P (2005). "Psoriatic arthritis: epidemiology, clinical features, course, and outcome". Annals of the Rheumatic Diseases. 64: ii14–ii17. doi:10.1136/ard.2004.032482. PMC 1766874. PMID 15708927.CS1 maint: multiple names: authors list (link)
Classification
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