Evidence map›Paper›PMID 35480068›Full record

ReviewOrthopedic research and reviews2022

Monitoring and Management of Fibrodysplasia Ossificans Progressiva: Current Perspectives.

Bernard J Smilde, Esmée Botman, Ruben D de Ruiter, Jan Maerten Smit, Berend P Teunissen, Wouter D Lubbers, Lothar A Schwarte, Patrick Schober, E Marelise W Eekhoff

Erratum issuedOpen access · goldAbstract readReview
In one paragraph

Review in Orthopedic research and reviews, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 24 papers.

0numbers the graph read from it
0cells of the map it votes in
24citing papers in PubMed
5.4field-weighted citation impact, top 4% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

24 citing papers in PubMed, 35 citations in OpenAlex.

  1. Article
  2. Article
  3. Review
  4. A cutaneous nodule revealing Münchmeyer's disease.Oxford medical case reports · 2025
    Article
  5. Review
  6. Article
  7. Article
  8. Article
  9. Article
  10. Review
  11. [Biomolecules · 2024
    Review
  12. Review
  13. Structural and molecular imaging-based characterization of soft tissue and vascular calcification in hyperphosphatemic familial tumoral calcinosis.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2024
    Article
  14. Article
  15. Review
  16. Review
  17. Article
  18. Article
  19. Context-dependent TGFβ family signalling in cell fate regulation.Nature reviews. Molecular cell biology · 2023
    Review
  20. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

9 authors at 1 institution in 1 country.

Bernard J SmildeAmsterdam UMC Location Vrije Universiteit Amsterdam, Department of Internal Medicine Section Endocrinology, Amsterdam, the Netherlands.ORCID 0000-0003-1473-6927
Esmée BotmanAmsterdam UMC Location Vrije Universiteit Amsterdam, Department of Internal Medicine Section Endocrinology, Amsterdam, the Netherlands.
Ruben D de RuiterAmsterdam UMC Location Vrije Universiteit Amsterdam, Department of Internal Medicine Section Endocrinology, Amsterdam, the Netherlands.
Jan Maerten SmitAmsterdam UMC, Amsterdam Bone Center, Amsterdam, the Netherlands.
Berend P TeunissenAmsterdam UMC, Amsterdam Bone Center, Amsterdam, the Netherlands.
Wouter D LubbersAmsterdam UMC, Amsterdam Bone Center, Amsterdam, the Netherlands.
Lothar A SchwarteAmsterdam UMC, Amsterdam Bone Center, Amsterdam, the Netherlands.
Patrick SchoberAmsterdam UMC, Amsterdam Bone Center, Amsterdam, the Netherlands.
E Marelise W EekhoffAmsterdam UMC Location Vrije Universiteit Amsterdam, Department of Internal Medicine Section Endocrinology, Amsterdam, the Netherlands.
Amsterdam University Medical Centers · NL

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Fibrodysplasia ossificans progressiva (FOP), sometimes known as myositis ossificans progressiva, is an ultra-rare disease in which bone is formed in muscular tissue, tendons and ligaments. This is known as heterotopic ossification (HO). FOP is caused by a heterozygous mutation in the highly conserved ACVR1/ALK2 gene which affects about 1 in 1.5-2 million individuals. At birth, patients with the predominant R206H mutation only exhibit a bilateral hallux valgus. During childhood, heterotopic bone formation develops in a typical pattern, affecting the axial muscles first before appendicular body parts are involved. HO can start spontaneously but is often elicited by soft tissue trauma or medical procedures. After soft tissue injury, an inflammatory process called a flare-up can start, followed by the formation of HO. HO leads to a limited range of motion, culminating in complete ankylosis of nearly all joints. As a result of HO surrounding the thorax, patients often suffer from thoracic insufficiency syndrome (TIS). TIS is the most common cause of a limited life expectancy for FOP patients, with a median life expectancy of 56 years. Management is focused on preventing soft-tissue injury that can provoke flare-ups. This includes prevention of iatrogenic damage by biopsies, intramuscular injections and surgery. Anti-inflammatory medication is often started when a flare-up occurs but has a poor basis of evidence. Several forms of potential treatment for FOP are being researched in clinical trials. Progression of the disease is monitored using CT and 18F-NaF PET/CT combined with functional assessments. Patients are regularly evaluated for frequently occurring complications such as restrictive lung disease. Here, we review the current management, monitoring and treatment of FOP.

Indexed as

activin A receptor type 1fibrodysplasia ossificans progressivaheterotopic ossificationtreatment strategies

Identifiers

PMID35480068
PMCPMC9035442
OpenAlexW4224236251

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.