Arthritis mutilans

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Arthritis mutilans
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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]

Contents

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]

Related Research Articles

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Arthritis is a term often used to mean any disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, swelling, and decreased range of motion of the affected joints. In some types of arthritis, other organs are also affected. Onset can be gradual or sudden.

<span class="mw-page-title-main">Rheumatoid arthritis</span> Type of autoimmune arthritis

Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involved, with the same joints typically involved on both sides of the body. The disease may also affect other parts of the body, including skin, eyes, lungs, heart, nerves, and blood. This may result in a low red blood cell count, inflammation around the lungs, and inflammation around the heart. Fever and low energy may also be present. Often, symptoms come on gradually over weeks to months.

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<span class="mw-page-title-main">Tietze syndrome</span> Inflammation, tenderness, and pain of the chest wall with swelling present

Tietze syndrome is a benign inflammation of one or more of the costal cartilages. It was first described in 1921 by German surgeon Alexander Tietze and was subsequently named after him. The condition is characterized by tenderness and painful swelling of the anterior (front) chest wall at the costochondral, sternocostal, or sternoclavicular junctions. Tietze syndrome affects the true ribs and has a predilection for the 2nd and 3rd ribs, commonly affecting only a single joint.

<span class="mw-page-title-main">Ankylosing spondylitis</span> Type of arthritis of the spine

Ankylosing spondylitis (AS) is a type of arthritis characterized by long-term inflammation of the joints of the spine, typically where the spine joins the pelvis. With AS, eye, bowel problems and back pain may occur. Joint mobility in the affected areas sometimes worsens over time. Ankylosing spondylitis is believed to involve a combination of genetic and environmental factors. More than 85% of people affected in the UK have a specific human leukocyte antigen known as the HLA-B27 antigen. The underlying mechanism is believed to be autoimmune or autoinflammatory. Diagnosis is based on symptoms with support from medical imaging and blood tests. AS is a type of seronegative spondyloarthropathy, meaning that tests show no presence of rheumatoid factor (RF) antibodies.

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<span class="mw-page-title-main">Psoriatic arthritis</span> Long-term inflammatory arthritis

Psoriatic arthritis (PsA) is a long-term inflammatory arthritis that occurs in people affected by the autoimmune disease psoriasis. The classic feature of psoriatic arthritis is swelling of entire fingers and toes with a sausage-like appearance. This often happens in association with changes to the nails such as small depressions in the nail (pitting), thickening of the nails, and detachment of the nail from the nailbed. Skin changes consistent with psoriasis frequently occur before the onset of psoriatic arthritis but psoriatic arthritis can precede the rash in 15% of affected individuals. It is classified as a type of seronegative spondyloarthropathy.

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<span class="mw-page-title-main">Rheumatoid nodule</span> Medical condition

A rheumatoid nodule is a lump of tissue, or an area of swelling, that appears on the exterior of the skin usually around the olecranon or the interphalangeal joints, but can appear in other areas. There are four different types of rheumatoid nodules: subcutaneous rheumatoid nodules, cardiac nodules, pulmonary nodules, and central nervous systems nodules. These nodules occur almost exclusively in association with rheumatoid arthritis. Very rarely do rheumatoid nodules occur as rheumatoid nodulosis in the absence of rheumatoid arthritis. Rheumatoid nodules can also appear in areas of the body other than the skin. Less commonly they occur in the lining of the lungs or other internal organs. The occurrence of nodules in the lungs of miners exposed to silica dust was known as Caplan’s syndrome. Rarely, the nodules occur at diverse sites on body.

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<span class="mw-page-title-main">Enteropathic arthropathy</span> Medical condition

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<span class="mw-page-title-main">Condylar resorption</span> Medical condition

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<span class="mw-page-title-main">Trabecular oedema</span> Medical condition

Trabecular edema, also known as bone marrow edema (BME), is a traditional term describing the interstitial fluid accumulation at the trabecular bone marrow. The term was first used in 1988, referring to the changes in the bone marrow due to inflammation. Bone marrow edema was later renamed to bone marrow lesion (BML), as later studies show that the increased fluid content in the trabecular bone was more likely caused by inflammatory responses instead of fluid influx. Hence, this narrows down the condition to the damage at the articular surface of the trabecular bones. Despite so, the terms BME and BML are still used interchangeably in radiology.

References

  1. ↑ National Institutes of Health Genetics Home Reference, Psoriatic arthritis. Accessed 2016-11-06.
  2. ↑ Nielsen, B.; Snorrason, E. (1 November 1946). "Arthritis Mutilans". Acta Radiologica. 27 (6): 607–616. doi:10.1177/028418514602700605. S2CID   81096498.
  3. ↑ Nelson, Louis S. (October 1938). "The opera-glass hand in chronic arthritis. 'La main en lorgnette' of Marie and Léri". JBJS. 20 (4): 1045–1049.
  4. ↑ Solomon, W. M.; Stecher, R. M. (1 September 1950). "Chronic Absorptive Arthritis or Opera-Glass Hand: Report of Eight Cases". Annals of the Rheumatic Diseases. 9 (3): 209–220. doi:10.1136/ard.9.3.209. PMC   1030771 . PMID   14783359.
  5. 1 2 3 4 Froimson, Avrum I. (October 1971). "Hand Reconstruction in Arthritis Mutilans: A CASE REPORT". The Journal of Bone & Joint Surgery. 53 (7): 1377–1382. doi:10.2106/00004623-197153070-00014. PMID   4939959.
  6. 1 2 Eisenstadt, H. B.; Eggers, G. W. N. (April 1955). "Arthritis Mutilans (Doigt, Main, Pied en Lorgnette)". The Journal of Bone & Joint Surgery. 37 (2): 337–346. doi:10.2106/00004623-195537020-00012.
  7. 1 2 3 4 Nograles, Kristine E; Brasington, Richard D; Bowcock, Anne M (February 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.
  8. 1 2 Sudoł-Szopińska, Iwona; Matuszewska, Genowefa; Kwiatkowska, Brygida; Pracoń, Grzegorz (20 February 2016). "Diagnostyka obrazowa łuszczycowego zapalenia stawów. Część I: etiopatogeneza, klasyfikacje i rentgenodiagnostyka" [Diagnostic imaging of psoriatic arthritis. Part I: etiopathogenesis, classifications and radiographic features]. Journal of Ultrasonography (in Polish). 16 (64): 65–77. doi: 10.15557/JoU.2016.0007 . PMC   4834372 . PMID   27104004.
  9. 1 2 3 4 Rodgers, M; Epstein, D; Bojke, L; Yang, H; Craig, D; Fonseca, T; Myers, L; Bruce, I; Chalmers, R; Bujkiewicz, S; Lai, M; Cooper, N; Abrams, K; Spiegelhalter, D; Sutton, A; Sculpher, M; Woolacott, N (February 2011). "Etanercept, infliximab and adalimumab for the treatment of psoriatic arthritis: a systematic review and economic evaluation". Health Technology Assessment . 15 (10): i–xxi, 1–329. doi: 10.3310/hta15100 . PMC   4781419 . PMID   21333232.
  10. 1 2 3 Tan, Yu M; Østergaard, Mikkel; Doyle, Anthony; Dalbeth, Nicola; Lobo, Maria; Reeves, Quentin; Robinson, Elizabeth; Taylor, William J; Jones, Peter B; Pui, Karen; Lee, Jamie; McQueen, Fiona M (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 Research & Therapy. 11 (1): R2. doi: 10.1186/ar2586 . PMC   2688232 . PMID   19126234.
  11. ↑ Eriksen, Erik F (25 November 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, Fiona M.; Benton, Nick; Perry, David; Crabbe, Jeff; Robinson, Elizabeth; Yeoman, Sue; McLean, Lachy; Stewart, Neal (July 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 & Rheumatism. 48 (7): 1814–1827. doi:10.1002/art.11162. PMID   12847674.
  13. ↑ Sudoł-Szopińska, Iwona; Pracoń, Grzegorz (30 June 2016). "Diagnostyka obrazowa łuszczycowego zapalenia stawów. Część II: rezonans magnetyczny i ultrasonografia" [Diagnostic imaging of psoriatic arthritis. Part II: magnetic resonance imaging and ultrasonography]. Journal of Ultrasonography (in Polish). 16 (65): 163–174. doi: 10.15557/JoU.2016.0018 . PMC   4954862 . PMID   27446601.
  14. ↑ Marzo-Ortega, H.; McGonagle, D.; Rhodes, L. A; Lyn Tan, A.; Conaghan, P. G; O'Connor, P.; Tanner, S. F; Fraser, A.; Veale, D.; Emery, P. (12 January 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. (April 2011). "Current Concepts in the Management of the Rheumatoid Hand". The Journal of Hand Surgery. 36 (4): 736–747. doi:10.1016/j.jhsa.2011.01.019. PMC   3086569 . PMID   21463736.
  16. ↑ Nalebuff, Edward A.; Garrett, John (December 1976). "Opera-glass hand in rheumatoid arthritis". The Journal of Hand Surgery. 1 (3): 210–220. doi:10.1016/s0363-5023(76)80040-8. PMID   1018089.
  17. ↑ Gladman, D D; Antoni, C; Mease, P; Clegg, DO; Nash, P (1 March 2005). "Psoriatic arthritis: epidemiology, clinical features, course, and outcome". Annals of the Rheumatic Diseases. 64 (suppl_2): ii14–ii17. doi: 10.1136/ard.2004.032482 . PMC   1766874 . PMID   15708927.
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