Clinical manifestations and pathogenesis of hydroxyapatite crystal deposition in juvenile dermatomyositis

Pachman, Lauren M.; Boskey, Adele L.

doi:10.1007/s11926-996-0031-5

Cited by 30 publications

(24 citation statements)

References 81 publications

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“…In dermatomyositis, dystrophic calcification is a well-recognized complication of inflammation within the skin or muscle, even though the precise precipitants of this condition are not clear. There is a strong consensus that dystrophic calcification is most likely to occur in patients whose dermatomyositis has been aggressive and persistent (13,14). Undertreatment is also considered to play a role in some cases.…”

Section: Discussionmentioning

confidence: 99%

Tumorous swelling on the back

Shrivastava¹,

Khanna²,

Metgud³

et al. 2011

Arthritis Care & Research

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A 29-year-old man with a painless mass on his back. History of the present illnessThe patient had noticed a painless mass over his right midback 2 years earlier. The mass had increased gradually in size. He had no history of fever, weight loss, anorexia, back pain, or similar lesions at any other site on his body. The overlying skin did not appear to be involved, and there was no history of any injury, injection, or surgical procedure on that site. There was no sensory loss or burning or tingling over or around the mass lesion, nor a history of chest pain, cough, hemoptysis, or breathlessness.Examination revealed a well-demarcated mass of moderate firmness that was approximately 7 ϫ 5 cm 2 in dimension. There was no erythema or tenderness to suggest inflammation. There was no spinal tenderness or gibbus, and scapular movements were not restricted. The remainder of the physical examination was unremarkable. Spine radiographs did not reveal any vertebral damage to suggest Pott's spine.Ultrasonography revealed a localized intramuscular abscess on the right scapular area. The patient was subsequently lost to followup for 1 year, during which time he underwent multiple incision and drainage procedures of the presumed abscess at other clinics. However, the lesion did not resolve. Cultures of the pus-like material exuded during abscess drainage were negative on several occasions.When the patient returned to our clinic, the mass had grown significantly in size and now occupied nearly twothirds of the right side of his back and the side of his trunk ( Figures 1A and B). The mass was firm, nontender, and nearly oblong, with few fluctuant areas. It was difficult to determine if the tumor was fixed to underlying structures, but the overlying skin was not tethered. Shoulder girdle movement and chest expansion were nearly normal. There were no enlarged regional lymph nodes. There was no fever and a complete review of systems was otherwise noncontributory. Medical historyThe patient had been diagnosed with dermatomyositis 7 years before presentation with the mass. His dermatomyositis had been characterized by polyarthritis, gradually progressive proximal muscle weakness, and ulcers over both elbows. There was no history of photosensitivity, malar rash, cough, dyspnea, dysphagia, or dysphonia. Examination had revealed a classic Gottron's sign over the dorsal surfaces of his metacarpal, proximal interphalangeal, and distal interphalangeal joints; punched out ulcers over both elbows; and proximal muscle weakness.The patient's muscle enzymes (creatine kinase, lactate dehydrogenase, and aspartate aminotransferase) were elevated more than 4-fold at presentation, accompanied by elevations in the erythrocyte sedimentation rate and C-reactive protein concentration. An enzyme immunoassay for antibodies to the human immunodeficiency virus was negative, as were assays for antinuclear antibodies and antiMi2 antibodies. Electrodiagnostic studies and the result of a muscle biopsy confirmed the diagnosis of dermatomyositis.The patient's elbow...

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Section: Discussionmentioning

confidence: 99%

Tumorous swelling on the back

Shrivastava¹,

Khanna²,

Metgud³

et al. 2011

Arthritis Care & Research

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“…These proteins, likely up-regulated in expression by tissue macrophages, cytokines and tissue injury, and may serve as a nidus for nucleation of the mineralized calcium that would then promote crystal growth [64,65]. Some of these proteins may initiate or promote mineralization, including dentin matrix protein 1, and matrix extracellular phosphoglycoprotein (MEPE), whereas others, including osteopontin, decorin, albumin and fetuin-A, apparently inhibit hydroxyapatite crystal growth [55,66]. Osteopontin apparently not only inhibits mineral deposition, but also actively promotes its dissolution by inducing expression of carbonic anhydrase in monocytes and promoting acidification of the extracellular environment [67].…”

Section: Pathogenesismentioning

confidence: 99%

The Presentation, Assessment, Pathogenesis, and Treatment of Calcinosis in Juvenile Dermatomyositis

et al. 2014

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Calcinosis is one of the hallmark sequelae of juvenile dermatomyositis (JDM), and despite recent progress in the therapy of JDM, dystrophic calcification still occurs in approximately one-third of patients. This review discusses our current, albeit limited, understanding of risk factors for the development of calcinosis in JDM, as well as approaches to assessment, and current views on its pathogenesis. Anecdotal approaches to treating calcinosis associated with JDM, including both anti-inflammatory therapies and agents aimed at inhibiting the deposition of calcium hydroxyapatite, are reviewed. An improved understanding of the pathogenesis of calcinosis, the establishment of standardized measurement tools to assess calcinosis, and randomized controlled trials employing more sensitive outcome measures are needed to develop efficacious therapies for this often disabling complication.

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“…It is believed that calcium salt deposits occur with severe cases of the disease with persistent inflammation 13 (generalized cutaneous vasculitis, muscle weakness and sustained muscle enzyme levels in elevation) and which do not respond to corticoid therapy. 8,10,[12][13][14][15][16] However, the majority of publications are just case reports.…”

Section: Introductionmentioning

confidence: 99%

Fatores de risco associados à calcinose na dermatomiosite juvenil

Sallum

Pivato

Doria-Filho

et al. 2008

J. Pediatr. (Rio J.)

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Objective: To identify risk factors associated with calcinosis in children and adolescents with juvenile dermatomyositis. Methods:A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale), pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies), gastrointestinal problems (gastroesophageal reflux) and/or heart disease (pericarditis and/or myocarditis); laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase); and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses. Conclusions:Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment.

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Clinical manifestations and pathogenesis of hydroxyapatite crystal deposition in juvenile dermatomyositis

Cited by 30 publications

References 81 publications

Tumorous swelling on the back

Tumorous swelling on the back

The Presentation, Assessment, Pathogenesis, and Treatment of Calcinosis in Juvenile Dermatomyositis

Fatores de risco associados à calcinose na dermatomiosite juvenil

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