|Ahead of print publication
Extensive calcinosis in a patient with raynaud's phenomenon and anti-ku antibody positivity
Kunal Chandwar, Digvijay Ekbote, Juhi Dixit, Kriti Kishor, Rasmi Ranjan Sahoo, Anupam Wakhlu
Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India
|Date of Submission||05-Sep-2021|
|Date of Acceptance||29-Sep-2021|
|Date of Web Publication||26-Nov-2021|
Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this URL:|
Chandwar K, Ekbote D, Dixit J, Kishor K, Sahoo RR, Wakhlu A. Extensive calcinosis in a patient with raynaud's phenomenon and anti-ku antibody positivity. Indian J Rheumatol [Epub ahead of print] [cited 2022 Jan 19]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=331251
A 41-year-old male with no known comorbidities presented with Raynaud's phenomenon and nodules over the fingertips and both thighs for the last 5 years. The nodules were multiple, small, nontender, hard in consistency and had increased in the last year [Figure 1]c. No other features of connective tissue disorder were present. Systemic examination was unremarkable. Investigations showed normal hemogram, renal and liver function tests, and inflammatory parameters. The metabolic profile (calcium, phosphate, alkaline phosphatase, and parathormone) was normal. X-ray of both hands [Figure 1]d, pelvis, and thighs [Figure 1]a showed extensive soft tissue calcification (orange arrows showing calcifications in gluteal, penile, and thigh regions). Computed tomography of both thighs showed multiple areas of fascial and intramuscular calcification in bilateral hip, groin, thigh, penile, and prepubic regions [Figure 1]b.
|Figure 1: (a) X-ray pelvis anteroposterior view showing extensive soft tissue calcification in gluteal, penile, and thigh regions (orange arrows). (b) Computed tomgoraphy scan of the thigh showing myositis ossificans of the thigh muscles. (c) Hands with calcinosis cutis (right second digit was amputated during a childhood accident) and (d) X-ray showing classical choky white deposits of calcinosis|
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Antinuclear antibody and extractable nuclear antigen profile were negative. Myositis panel revealed anti-Ku antibodies. He had previously received steroids, methotrexate, diltiazem, aluminium hydroxide, alendronate, and two infusions of zoledronic acid without significant relief. Intravenous pamidronate was administered at 1 mg/kg weight (maximum 60 mg) for 3 consecutive days. Follow-up at 6 months showed no further progression of calcinosis.
Herein, we present a case of extensive, progressive, treatment-resistant dystrophic calcification in a middle-aged male patient who had long-standing Raynaud's without internal organ involvement and isolated anti-Ku antibody was positive. Calcinosis is associated with a variety of disorders including autoimmune diseases. Among the autoimmune disorders, localized calcinosis is typically seen in systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) whereas widespread depositions are observed in juvenile dermatomyositis.
Although calcinosis in the setting of anti-Ku antibodies has been reported in SSc–polymyositis overlap, it is extremely rare in other autoimmune disorders such as SLE and idiopathic inflammatory myopathies (IIMs). Phenotypic manifestations are known to differ in IIM among different ethnic groups. The isolated association of anti-Ku antibodies with extensive calcification is neither described in Indian myositis patients nor in other international cohorts. Calcinosis is associated with certain autoantibody types, and newer antibodies continue to be described over the years. Analyzing sera of such patients with the immunoprecipitation assay may open the door to the identification of associated newer myositis-specific autoantibodies in the future.
From a management standpoint, this case highlights aggressive calcinosis in anti-Ku–positive IIM and the utility of pamidronate in the treatment of the condition.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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