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Case report


Camptodactyly-arthropathy-coxa vara-pericarditis syndrome: important differential for juvenile idiopathic arthritis.

Ritu Manoj KakkarSameer SonejiRashmi R BadheShrinivas B Desai
Department of Radiology, Jaslok Hospital and Research Centre, Global Hospital, Mumbai, Maharashtra, India
Date of Submission: 10-Feb-2013, Date of Acceptance: 14-May-2013, Date of Web Publication: 29-Jun-2013.
Corresponding Author:
Corresponding Author

Ritu Manoj Kakkar

Department of Computed Tomography Scan/Magnetic Resonance Imaging, Jaslok Hospital and Research Center, 15 Dr. G Deshmukh Marg, Mumbai ‑ 400 026, Maharashtra, India.
E-mail: riturupesh@gmail.com

Corresponding Author:
Corresponding Author

Ritu Manoj Kakkar

Department of Computed Tomography Scan/Magnetic Resonance Imaging, Jaslok Hospital and Research Center, 15 Dr. G Deshmukh Marg, Mumbai ‑ 400 026, Maharashtra, India.
E-mail: riturupesh@gmail.com

DOI: 10.4103/2156-7514.114211 Facebook Twitter Google Linkedin

ABSTRACT


Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is an inherited disorder characterized by congenital or early-onset flexion camptodactyly, childhood-onset of non-inflammatory arthropathy, often associated with non-inflammatory pericarditis or pericardial effusion and progressive coxa vara. The causative gene is located on chromosome band 1q25-31. This gene encodes for "proteoglycan-4" (PRG-4), which is a surface lubricant for joints and tendons. This syndrome has distinct radiological and histological features, which are important to recognize since it may clinically mimic juvenile idiopathic arthritis and mutation studies may not be easily available. We describe a case of a 3-year 3-month-old female with features of CACP syndrome.
Keywords: Camptodactyly-arthropathy-coxa Vara-pericarditis Syndrome, Intraosseous Fluid-filled Herniations, Juvenile Idiopathic Arthritis, No Joint Erosions

Cited in 4 Documents

  1. Yasser Emad, Yasser Ragab, Osama Ibrahim, Maher Khalifa, Ahmed Dawood and Johannes J. Rasker (2017) Spinal involvement in Camptodactyly Arthropathy Coxa-vara Pericarditis (CACP) syndrome in two Yemeni sisters. The Egyptian Rheumatologist 39(3):195. doi: 10.1016/j.ejr.2016.10.001
  2. G. Keyßer, C. Schäfer, H. Zeidler, A. Rubbert-Roth and B. Michel (2019) Differenzialdiagnose rheumatischer Erkrankungen. (Chapter 7):105. doi: 10.1007/978-3-662-56575-9_7
  3. Bram Peters, Janneke H. M. Schuurs-Hoeijmakers, Joris Fuijkschot, Annette Reimer, Michiel van der Flier, Dorien Lugtenberg and Esther P.A.H. Hoppenreijs (2016) Protein-losing enteropathy in camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome. Pediatr Rheumatol 14(1):. doi: 10.1186/s12969-016-0093-5
  4. Florent Eymard and Xavier Chevalier (2018) Fausses arthroses primaires. Revue du Rhumatisme Monographies 85(4):295. doi: 10.1016/j.monrhu.2018.05.001

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