Diagnostic Criteria for Temporomandibular Disorders (2014)

Overview The Diagnostic Criteria for Temporomandibular Disorders (DC/TMD), by Schiffman et al, are available in the Journal of Oral & Facial Pain and Headache, 2014.  The DC/TMD is intended for use in both clinical settings and applied research settings.  Schiffman et al describe the rationale and methodology underlying the changes from the RDC/TMD to the DC/TMD.  The extensive development process can be explored in greater detail. Further research examining core properties is expected, and forthcoming additional DC/TMD research tools are intended to augment the DC/TMD for such research.  All tools required for clinical implementation are available on this page.  The DC/TMD tools are living documents, and revisions are managed by the Consortium Network; the most current version will always be available here.  Translations Early translated versions (Dutch, Spanish, and Swedish) of the DC/TMD were developed in order to test the adequacy of the English source documents and some of these versions have also been used in examiner training, calibration, and reliability field trials; these versions will soon be available for download.  Consortium members have begun work on a total of 27 translations: Arabic, Chinese (short form), Danish, Estonian, Farsi, Finnish, French, German, Greek, Hebrew, Hindi, Indonesian, Italian, Japanese, Korean, Malay, Nepali, Norwegian, Polish, Portuguese (Brazil), Portuguese (Portugal), Thai, Turkish, and Urdu. Please contact the team leaders on this list for more information about a translation, and please contact us if your language of interest is not yet included and you would like to request permission to do a translation. Please see theTranslation Guidelines page for further information regarding translation procedures for the Consortium.   Examiner Training, Calibration, and Reliability Assessment An annotated video of the DC/TMD clinical examination has been completed and is currently under peer review at Med Portal.  A link will be posted here for accessing the video once it is published.  Self-study guidelines as well as description of supervised skill development are described in further detail. Additional information will be provided at a later time regarding scheduled workshops for examiner training. Documents for Downloading The URL links in the references of the DC/TMD publication access resource documents on this website.  If the "Modified Date" in the list to the right is prior to January 29, 2014, the document version is current as of the DC/TMD publication.  Document filenames contain dates representing the relative version of the document; however, those filename dates may not correspond to the "Modified Date" shown tin the file listing.  The inconsistency emerges due to separate production cycles, in the final month, for the manuscript and for the support documents.  All documents contain a date within, usually in the footer; the internal date corresponds to the Modified Date in the listing.  The DC/TMD reference URLs will always link to the documents current at the time of publication.  Any Modified Date in the file listing after January 29, 2014 represents an updated version since the DC/TMD was published, and this page will always contain the most current version.  Suggestions for improving this arrangement are welcomed. Complete DC/TMD Instrument Set Document Link Modified Date DC/TMD: Complete PDF Open 1/29/2014 Individual Instruments, Forms, Protocol Document Link Modified Date TMD Pain Screener Open 10/11/2013 DC/TMD Symptom Questionnaire Open 5/12/2013 Demographics Open 5/12/2013 Examination form: International Open 5/1/2/2013 Examination form: North America Open 5/1/2/2013 Examiner Protocol Open 1/24/2014 DC/TMD Decision Trees Open 1/24/2014 DC/TMD DIagnostic Criteria Open 1/24/2014 Pain drawing Open 5/12/2013 Graded Chronic Pain Scale (v2) Open 5/12/2013 JFLS-8 Open 5/12/2013 JFLS-20 Open 5/12/2013 PHQ-4 Open 5/12/2013 PHQ-9 Open 5/12/2013 GAD-7 Open 5/12/2013 PHQ-15 Open 5/12/2013 Oral Behaviors Checklist Open 5/12/2013

Criteria for the Diagnosis of Asthma

Asthma is a chronic inflammatory disease of the airways that is characterized by variable narrowing of the airways and symptoms of intermittent dyspnea, wheezing, and nighttime or early-morning coughing.

Criteria for the Diagnosis of Asthma

Presence of episodic symptoms of airflow obstruction or airway hyperresponsiveness
Objective assessment consisting of one of the following

Airflow obstruction that is at least partially reversible with the use of an inhaled short-acting beta 2-agonist, as shown by one of three variables

An increase in FEV1 of >/=12% from baseline
An increase in predicted FEV1 of >/=10 percentage points from baseline
An increase in PEF of >/=20% (or 60 liters/min) from baseline

Diurnal variation in PEF (measured twice daily) of more than 10%

* FEV1 denotes forced expiratory volume in 1 second, and PEF peak expiratory flow.

References:

1. Bel EH. Clinical Practice. Mild asthma. N Engl J Med. 2013 Aug 8;369(6):549-57.[Medline]
2. Mintz M. Asthma update: part I. Diagnosis, monitoring, and prevention of disease progression. Am Fam Physician. 2004;70:893-898. [Medline]

Intravenous Immune Globulin in Autoimmune and Inflammatory Diseases

Initially used for the treatment of immunodeficiencies, intravenous immunoglobulins (IVIg) have increasingly been used as immunomodulatory agent in autoimmune and inflammatory disorders. The mode of action of IVIg is enigmatic, probably involving Fc-dependent and/or F(ab')2-dependent non-exclusive mechanisms of action. IVIg broadly interacts with the different components of the immune system: cytokines, complement, Fc receptors and several cell surface immunocompetent molecules. IVIg also has an impact on effector functions of immune cells. These mechanisms of action of IVIg reflect the importance of natural antibodies in the maintenance of immune homeostasis.

Diseases for Which Intravenous Immune Globulin Has Been Shown to Be Beneficial

FDA-approved indications

• Primary immunodeficiency disease
• Chronic lymphocytic leukemia
• Pediatric HIV infection
• Kawasaki’s disease
• Allogeneic bone marrow transplantation
• Chronic inflammatory demyelinating polyneuropathy
• Kidney transplantation involving a recipient with a high antibody titer or an ABO-incompatible donor
• Multifocal motor neuropathy

Additional approved indications with criteria

Neuromuscular disorders

• Guillain–Barré syndrome
• Relapsing–remitting multiple sclerosis
• Myasthenia gravis
• Refractory polymyositis
• Polyradiculoneuropathy
• Lambert–Eaton myasthenic syndrome
• Opsoclonus–myoclonus
• Birdshot retinopathy
• Refractory dermatomyositis

Hematologic disorders

• Autoimmune hemolytic anemia
• Severe anemia associated with parvovirus B19
• Autoimmune neutropenia
• Neonatal alloimmune thrombocytopenia
• HIV-associated thrombocytopenia
• Graft-versus-host disease
• Cytomegalovirus infection or interstitial pneumonia in patients undergoing bone marrow transplantation

Dermatologic disorders

• Pemphigus vulgaris
• Pemphigus foliaceus
• Bullous pemphigoid
• Mucous-membrane (cicatricial) pemphigoid
• Epidermolysis bullosa acquisita
• Toxic epidermal necrolysis or Stevens–Johnson syndrome
• Necrotizing fasciitis

* This is an abbreviated list of conditions approved under Medicare Part D or Aetna Clinical Policy Bulletin (2012). Criteria include medical certainty of diagnosis, medical necessity owing to the failure of usual treatments, contraindications to usual treatments, rapid progression or relapse, documentation of progress, and attempts to adjust drug dosages without improvement. FDA denotes Food and Drug Administration, and HIV human immunodeficiency virus.

References:

1. Gelfand EW. Intravenous immune globulin in autoimmune and inflammatory diseases. N Engl J Med. 2012 Nov 22;367(21):2015-25. [Medline]
2. Jolles S, Sewell WA, Misbah SA. Clinical uses of intravenous immunoglobulin. Clin Exp Immunol. 2005 Oct;142(1):1-11. [Medline]

Diagnostic Criteria for Chronic HBV Infection

Chronic HBV infection is a necroinflammatory disease of the liver caused by persistent infection with HBV, and can be categorized as hepatitis B e antigen (HBeAg) positive or negative. Inactive hepatitis B surface antigen (HBsAg) carriers have HBV infection of the liver without significant, ongoing necroinflammatory disease. HBV infection is resolved when there is no further virologic, biochemical, or histologic evidence of active viral infection or disease.

Diagnostic criteria related to HBV include the following:

• Chronic hepatitis B: HBsAg positive for more than six months, serum HBV DNA greater than 20,000 IU per mL (lower values of 2,000 to 20,000 IU per mL often occur with HBeAg-negative chronic hepatitis B), persistent or intermittent elevation in alanine transaminase (ALT) or aspartate transaminase (AST) levels, and liver biopsy showing chronic hepatitis with moderate or severe necroinflammation.
• Inactive HBsAg carrier state: HBsAg positive for more than six months, HBeAg negative and anti-HBeAg positive, serum HBV DNA less than 2,000 IU per mL, persistently normal ALT and AST levels, liver biopsy confirming absence of significant hepatitis.
• Resolved hepatitis B: known history of acute or chronic hepatitis B or the presence of anti-hepatitis B core antigen (anti-HBcAg) with or without anti-HBsAg, HBsAg negative, undetectable serum HBV DNA (very low levels may be detectable with sensitive prostate-specific antigen assays), and normal ALT levels.

References:

1. Kuo A, Gish R. Chronic hepatitis B infection. Clin Liver Dis. 2012 May;16(2):347-69.[Medline]
2. Liaw YF, Chu CM. Hepatitis B virus infection. Lancet. 2009;373:582–92. [Medline]
3. Bonino F, Piratvisuth T, Brunetto MR, Liaw YF. Diagnostic markers of chronic hepatitis B infection and disease. Antivir Ther. 2010;15 Suppl 3:35-44. [Medline]

Revised Diagnostic Criteria for Rett Syndrome (RTT)

Rett syndrome (RTT) is a severe neurodevelopmental disease that affects approximately 1 in 10,000 live female births and is often caused by mutations in Methyl-CpG-binding protein 2 (MECP2).

Revised diagnostic criteria for RTT.

RTT Diagnostic Criteria 2010

• Consider diagnosis when postnatal deceleration of head growth observed.

Required for typical or classic RTT

1. A period of regression followed by recovery or stabilization*
2. All main criteria and all exclusion criteria
3. Supportive criteria are not required, although often present in typical RTT

Required for atypical or variant RTT

1. A period of regression followed by recovery or stabilization*
2. At least 2 out of the 4 main criteria
3. 5 out of 11 supportive criteria

Main Criteria

1. Partial or complete loss of acquired purposeful hand skills.
2. Partial or complete loss of acquired spoken language**
3. Gait abnormalities: Impaired (dyspraxic) or absence of ability.
4. Stereotypic hand movements such as hand wringing/squeezing, clapping/tapping, mouthing and washing/rubbing automatisms

Exclusion Criteria for typical RTT

1. Brain injury secondary to trauma (peri- or postnatally), neurometabolic disease, or severe infection that causes neurological problems***
2. Grossly abnormal psychomotor development in first 6 months of life#

Supportive Criteria for atypical RTT##

1. Breathing disturbances when awake
2. Bruxism when awake
3. Impaired sleep pattern
4. Abnormal muscle tone
5. Peripheral vasomotor disturbances
6. Scoliosis/kyphosis
7. Growth retardation
8. Small cold hands and feet
9. Inappropriate laughing/screaming spells
10. Diminished response to pain
11. Intense eye communication - “eye pointing”

*Because MECP2 mutations are now identified in some individuals prior to any clear evidence of regression, the diagnosis of “possible” RTT should be given to those individuals under 3 years old who have not lost any skills but otherwise have clinical features suggestive of RTT. These individuals should be reassessed every 6–12 months for evidence of regression. If regression manifests, the diagnosis should then be changed to definite RTT. However, if the child does not show any evidence of regression by 5 years, the diagnosis of RTT should be questioned.

**Loss of acquired language is based on best acquired spoken language skill, not strictly on the acquisition of distinct words or higher language skills. Thus, an individual who had learned to babble but then loses this ability is considered to have a loss of acquired language.

***There should be clear evidence (neurological or ophthalmological examination and MRI/CT) that the presumed insult directly resulted in neurological dysfunction.

#Grossly abnormal to the point that normal milestones (acquiring head control, swallowing, developing social smile) are not met. Mild generalized hypotonia or other previously reported subtle developmental alterations during the first six months of life is common in RTT and do not constitute an exclusionary criterion.

##If an individual has or ever had a clinical feature listed it is counted as a supportive criterion. Many of these features have an age dependency, manifesting and becoming more predominant at certain ages. Therefore, the diagnosis of atypical RTT may be easier for older individuals than for younger. In the case of a younger individual (under 5 years old) who has a period of regression and >/=2 main criteria but does not fulfill the requirement of 5/11 supportive criteria, the diagnosis of “probably atypical RTT” may be given. Individuals who fall into this category should be reassessed as they age and the diagnosis revised accordingly.

References:

1. Jeffrey L. Neul, Walter E. Kaufmann, Daniel G. Glaze, John Christodoulou, Angus J. Clarke, Nadia Bahi-Buisson, Helen Leonard, Mark E. S. Bailey, N. Carolyn Schanen, Michele Zappella, Alessandra Renieri, Peter Huppke, Alan K. Percy. Rett Syndrome: Revised Diagnostic Criteria and Nomenclature. Ann Neurol. 2010 December; 68(6): 944–950. [Medline]
2. Percy AK, Neul JL, Glaze DG, Motil KJ, Skinner SA, Khwaja O, Lee HS, Lane JB, Barrish JO, Annese F, McNair L, Graham J, Barnes K. Rett syndrome diagnostic criteria: lessons from the Natural History Study. Ann Neurol. 2010 Dec;68(6):951-5. [Medline]
3. Huppke P, Gärtner J. Molecular diagnosis of Rett syndrome. J Child Neurol. 2005 Sep;20(9):732-6. [Medline]

Diagnostic Criteria for Marfan Syndrome (MFS)

Marfan syndrome is currently diagnosed using criteria based on an evaluation of the family history, molecular data, and 6 organ systems.

Diagnostic Criteria for Marfan Syndrome (MFS)


The Ghent criteria consist of major and minor criteria. The major criteria are features or symptoms that are common in people with Marfan syndrome and rare in people who do not have the syndrome.

Minor criteria are features or symptoms that are present in people with Marfan syndrome, but are also present in people who do not have it.

To be diagnosed with Marfan syndrome using the Ghent criteria, you must have a number of different criteria as described below.

If you have a family history of Marfan syndrome, you will need to have one of the major criteria and one of the minor criteria that affect different systems in your body, such as your skeleton and your blood vessels.
If you do not have a family history of Marfan syndrome, you will need to have two major criteria and one of the minor criteria that affect different systems in your body.

ILAR Classification Criteria for Juvenile Idiopathic Arthritis (JIA)

Juvenile idiopathic arthritis is a heterogeneous group of diseases characterised by arthritis of unknown origin with onset before age of 16 years.

International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA)

JIA can be diagnosed if age at onset is under 16 years, disease duration is 6 weeks or greater, and other known conditions are excluded.

• Systemic-onset JIA: Systemic arthritis is diagnosed if there is arthritis in 1 or more joints with, or preceded by, fever of at least 2 weeks' duration. Signs or symptoms must have been documented daily for at least 3 days and accompanied by 1 or more of the following: evanescent rash, generalised lymphadenopathy, hepato/splenomegaly, serositis. (Exclusions are A, B, C, and D from the exclusion list below.)
• Persistent or extended oligoarthritis: Oligoarthritis is diagnosed if there is arthritis affecting 1 to 4 joints during the first 6 months. Persistent oligoarthritis affects up to 4 joints throughout the course of the disease, and extended oligoarthritis affects more than 4 joints after the first 6 months of disease. (Exclusions are A, B, C, D, and E from the exclusion list below.)
• RF-negative polyarthritis: Polyarthritis (RF-negative) is diagnosed if there is rheumatoid factor (RF)-negative arthritis affecting 5 or more joints during the first 6 months of disease. (Exclusions are A, B, C, D, and E from the exclusion list below.)
• RF–positive polyarthritis: Polyarthritis (RF-positive) is diagnosed if there is RF-positive arthritis affecting 5 or more joints during the first 6 months of disease. Two or more RF tests (taken at least 3 months apart) are positive during the first 6 months of disease. (Exclusions are A, B, C, and E from the exclusion list below.)
• Psoriatic JIA: Psoriatic arthritis is diagnosed if there is arthritis and psoriasis, or arthritis and at least 2 of the following: dactylitis, nail pitting, onycholysis, and/or family history of psoriasis (in a first-degree relative). (Exclusions are B, C, D, and E from the exclusion list below.)
• Enthesitis-related arthritis: Enthesitis-related arthritis is diagnosed if there is arthritis and/or enthesitis with at least 2 of the following: presence or history of sacroiliac joint tenderness with or without inflammatory lumbosacral pain; presence of HLA B27 antigen; onset of arthritis in a male over 6 years of age; acute (symptomatic) anterior uveitis; history of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, Reiter's syndrome, or acute anterior uveitis in a first-degree relative. (Exclusions are A, D, and E from the exclusion list below.)
• Undifferentiated: Undifferentiated arthritis is diagnosed if there is arthritis that does not fulfil criteria in any of the above categories or that fulfils criteria for 2 or more of the above categories.