Rare Rheumatology News

Disease Profile


Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.


US Estimated

Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)



Trochleitis is the swelling of structures in the eye that help control eye movement, specifically the oblique tendon and surrounding tissues.[1][2][3] Trochleitis may be isolated (occur alone or with migraine) or develop in association with an inflammatory condition, such as lupus or arthritis.[1][4][5][2] Signs and symptoms include aching and/or stabbing pain in the inner orbit of the eye (e.g., near the nose and inner eyebrow).[4] The pain tends to worsen when looking up and down, and when gentle pressure is applied.[3] Trochleitis usually affects a single eye, but can involve both eyes. Ultrasoundimaging and noting a rapid improvement with locally injected corticosteroids aids in diagnosis.[4][2] Treatment may involve oral or locally injected corticosteroids.[3][2] Long-term outlook is good with symptoms resolving within weeks to months in most cases.[3]


Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • The Mayo Clinic provides information on eye pain in general and may be a helpful resource. Click on Mayo Clinic to view the information.
    • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.


      1. Gutmark R, Eliasieh K, Rivera-Michlig R. A case of bilateral trochleitis in adult-onset Still's disease. Semin Arthritis Rheum. April 2014; 43(5):689-91. https://www.ncbi.nlm.nih.gov/pubmed/24176730. Accessed 5/28/2014.
      2. Yangüela J, Pareja JA, Lopez N, Sánchez Del Río M. Trochleitis and migraine headache. Neurology. 2002 Mar 12; 8(5):802-5. https://www.ncbi.nlm.nih.gov/pubmed/11889246. Accessed 5/28/2014.
      3. Tychsen L, Tse DT, Ossoinig K, Anderson RL. Trochleitis with superior oblique myositis. Ophthalmology. September 1984; 91(9):1075-9. https://www.ncbi.nlm.nih.gov/pubmed/6387570. Accessed 5/28/2014.
      4. Fonseca P, Manno RL, Miller NR. Bilateral sequential trochleitis as the presenting feature of systemic lupus erythematosus. J Neuroophthalmol. March 2013; 33(1):74-6. https://www.ncbi.nlm.nih.gov/pubmed/23114286. Accessed 5/28/2014.
      5. Zaragoza-Casares P, Gómez-Fernández T, Gómez de Liaño MA, Zaragoza-Garcia P. Bilateral idiopathic trochleitis as a cause of frontal cephalgia. Headache. March 2009; 49(3):476-7. https://www.ncbi.nlm.nih.gov/pubmed/19267791. Accessed 5/28/2014.