List of General Autoinflammatory/Periodic Fever Syndrome Studies and References

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This is a collection of research and information on various autoinflammatory diseases. Each one covers multiple fever syndromes in one article, which is helpful to use to compare symptoms seen in a patient to symptoms seen in each of these conditions. Several include lab tests to consider and treatment options.

Autoinflammatory Diseases Online Database – This online database compares symptoms and lab results of dozens of autoinflammatory diseases in an easy to read chart format.

Recommendations for the management of autoinflammatory diseases – This gives a good overview of how to diagnose and treat CAPS, TRAPS, and MKD (HIDS and MA).

Untangling the Web of Systemic Autoinflammatory Diseases – Good up to date information, as of 2014, on several periodic fever syndromes. Includes information on some of the newer classified autoinflammatory diseases. Covers familial Mediterranean fever (FMF), mevalonate kinase deficiency syndrome (MKD/HIDS), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), NLRP12-associated autoinflammatory disorder (NLRP12-AD), Blau syndrome, pigmentary hypertrichosis and nonautoimmune insulin-dependent diabetes mellitus syndrome (PHID/SLC29A3) and more.

Hereditary Periodic Fever Syndromes – Written by Dr. Kastner, this covers FMF, TRAPS, HIDS, NOMID. Explains differences between TRAPS and the other similar syndromes.

Rash Pattern and Duration Distinguish Hereditary Periodic Fever Syndromes – By Dr. Kastner. Explains the differences in rashes of TRAPS, HIDS, FMF, and CAPS. The TRAPS rash is migratory.

Autoinflammatory syndromes: Fever is not always a sign of infection – From Doctors Zeft and Spalding at the Cleveland Clinic. Good diagrams of the biological pathways of TRAPS, Blau, CAPS, FMF, HIDS, and PAPA. Differences between TRAPS and FMF or HIDS are discussed.

Autoinflammatory syndromes: diagnosis and management – a good synopsis of FMF, HIDS, TRAPS, CAPS, Blau Syndrome, PAPA Syndrome, PFAPA, and Majeed’s Syndrome. Gives information on differences in the diseases and best treatments as of 2010.

The autoinflammatory diseases – Published in 2012, this also covers FMF, MVK Deficiency (HIDS), TRAPS, CAPS,  NLRP 12 mutations, Blau Syndrome, PAPA Syndrome, Majeed’s, DIRA, and PFAPA.

The Expanding Spectrum of Systemic Autoinflammatory Diseases: Misadventures in the Genomics of Inflammation – More information on the different fever disorders. From a lecture given by Dr. Kastner, has a lot of details on individual cases.

Clinical Immunology Review Series: An approach to the patient with a periodic fever syndrome – Covers FMF, TRAPS, HIDS, CAPS, DIRA, PAPA, BLAU, PFAPA, and Schnitzeler’s

Treatment of hereditary autoinflammatory diseases – Video/Powerpoint presentation of treatment options and results. Focuses primarily on FMF, CAPS, HIDS, and TRAPs.

Autoinflammatory vs. Autoimmune – It depends on if it’s the innate or the adaptive immune system that’s malfunctioning whether some diseases are considered autoinflammatory or autoimmune. Learn the differences here.

Understanding Autoiflammatory Diseases – Autoinflammatory diseases defined by the National Institutes of Health (NIH). FMF, NOMID, TRAPS, DIRA, and Behcet’s are discussed.

Childhood versus adulthood-onset autoinflammatory disorders: myths and truths intertwined – Research from several research centers in Italy. This study looks at differences in the age of onset for various fever syndomes. Diseases covered include, FMF, familial Mediterranean fever; TRAPS, tumor necrosis factor receptor-associated periodic syndrome; HIDS, hyper-gammaglobulinemia D syndrome; FCAS, familial cold autoinflammatory syndrome; MWS, Muckle-Wells syndrome; CINCAs, chronic infantile neurologic cutaneous articular syndrome; NLRP12AD, NLRP12-associated autoinflammatory disorder; BS, Blau syndrome. Click the “Download This PDF File” at the top to get a full view of this PDF.

Autoinflammatory Syndromes at DermNet NZ – This has a good overview of several conditions with a focus on the types of rashes and skin biopsy results that come with each. Includes information on FMF, TRAPS, HIDS, CAPS, PAPA syndrome, Blau, DIRA, mevalonic aciduria (MA), Majeed syndrome, Schnizler syndrome, Behcet’s disease, and Still’s disease.

How not to miss autoinflammatory diseases masquerading as urticarial – A look at severe autoinflammatory diseases from a dermatology perspective. Urticarial rashes are common in several. This study presents “clinical clues and tips that can help to identify autoinflammatory disorders in patients presenting with chronic urticarial rash and discuss their clinical picture and management.” Includes Still’s disease, CAPS, FMF, HIDS, MAS, PFAPA, soJIA, Schnitzler’s syndrome, TRAPS, FCAS2, and other conditions. Has several good photos of rashes that can occur in these periodic fever syndromes.

 

Care Packages & Birthday Club for Kids with Autoinflammatory Diseases

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CrateImageNetUPDATE: This program has been completed and sign ups ended on January 30th, 2015.  We have other things planned for the future, so please sign up to follow our blog, and like us on facebook to get the latest updates on programs.

Crate Expectations Care Packages

The Autoinflammatory Alliance is pleased to announce our new program for children with autoinflammatory diseases. Crate Expectations is a care package program in which we support our community members by sending unique care packages that will contain items intended to uplift and encourage.

If you are not sure what autoinflammatory diseases are, please look over this page and also read this post about the difference between autoinflamatory diseases and autoimmune diseases. Please be aware that we are a non-profit organization helping patients with autoinflammatory diseases, and do not have the resources to open this program to other conditions that are not autoinflammatory diseases.

Each care package will be created based upon the completed form (also below). The items will vary but will potentially include activities/crafts, lip balms/chapstick, hats and gloves, and a few other feel good items. The Crate Expectation package will be free of charge and limited to 1 per person with an autoinflammatory disease or periodic fever syndrome per calendar year. This program is funded through an ABC Summer of Service Youth Serve Grant. If you have any questions or comments, please use the contact form.

NOTE: We will be shipping the care packages in February 2015, in honor of Rare Disease Day.  You must register your child by 11:59 pm on January 30, 2015 to be included in this year’s care package program. Also, we will only be able to ship care packages to children with a systemic autoinflammatory disease (list is in the form-please check off what is applicable), and regret that we cannot support care packages for other diseases, as the grant funding was specifically for autoinflammatory disease patients. We are a small non-profit trying to make a big difference for our patients, and also support awareness and research. If you have juvenile arthritis, or other such conditions that are not specifically autoinflammatory diseases, please visit the Arthritis Foundation or other charities to enroll in other programs that may be appropriate for your child.

We have had a tremendous level of interest in this care package program! If you can donate to help with the costs for shipping, please donate at the link below. Please contact us if you would like to sponsor, or donate items for the care packages. Thank you!


BirthdayClubNetFirst Aid Smiles Birthday Club

The Flying Lifeguards is pleased to present “First Aid Smiles,” a birthday club for children who have an autoinflammatory disease. Fever, rash, and systemic inflammation are often a daily event which means even birthdays and holidays are affected by these diseases. The Flying Lifeguards want to send your child a First Aid Smile birthday card to make their special day a brighter one. Please sign up no later than 2 weeks prior to their birthday to allow enough time for a card to be sent. This is also a free program to the recipients. We have partnered with them for the sign up for this program on the same form.

Your contact info and details will remain private, and not shared with anyone outside of the Autoinflammatory Alliance, or for the purpose of sending a care package or the birthday club packs from The Flying Lifeguards.

Click here to learn more about autoinflammatory diseases and how they differ from autoimmune.

Autoinflammatory diseases are also known as periodic fever syndromes. Click here to learn what is a periodic fever syndrome.

The Autoinflammatory Alliance is a nonprofit organization dedicated to helping those with autoinflammatory diseases.

Donate now to help with awareness, education, and research for these rare diseases.




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ShotBlocker and the Buzzy Bee for Shots Reviews – Do These Devices Stop Injection Pain?

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We’ve tried out both the ShotBlocker and the Buzzy Bee for shots. Both work surprisingly well at reducing the pain of an injection according to our 5-year-old. Most importantly, our 5-year-old asks us to use these when getting shots for his condition.

Both work on the same principal that distraction reduces pain. By distracting the brain with the ShotBlocker and/or Buzzy Bee, you take the brain’s focus away from the pain and your child may not even notice the injection.

To use either of these devices, you do have to work quickly. The distraction does not last long. Have your shot ready to go and then place the device in position just before you give the shot for best results.

Buzzy Bee for ShotsThe Buzzy Bee for Shots

The Buzzy Bee Pain Relief System uses vibration and cold to take the brain’s attention away from the shot. It comes with a small ice pack that attaches to the Buzzy to place against the skin. You then turn on the Buzzy and it vibrates. You place the Buzzy somewhere between the shot and the brain to disrupt the pain signals going to the brain. For example, when giving the shot in the leg, we place the Buzzy higher up on the thigh than where we give the shot.

We do not use the ice pack part of the Buzzy. We just use the Buzzy for the vibrations because my son doesn’t want anything cold touching him. For patients with CAPS or other autoinflmmatory diseases that have cold as a trigger, know that the ice pack is optional, removable, and not essential for this device to function.

Click here to buy the Buzzy on Amazon.

See how to use the Buzzy Bee for shots in this video:

reducing pain of injectionsThe ShotBlocker

This is the first one we tried and we all were impressed. I was surprised that first time we used it how long it took before he noticed the injection happening. It was only a few seconds, but since medications used to treat fever syndromes are extremely painful, that few seconds makes a big difference. The underside that you press onto the skin has small plastic rounded nubs. When pressed gently into the skin, the brain focuses on the odd sensation rather than the injection. The center cut out gives you the perfect place to inject.

The ShotBlocker does take some practice using. A couple of times he told us we pressed too hard and it was painful. The ShotBlocker should not hurt. The nubs on the bottom should only be distracting, or maybe a little annoying to the brain.

Click here to buy the ShotBlocker on Amazon.

Which One is Best?

Overall, I highly recommend both devices. In fact, our son likes us to use both at the same time. This is possible to use both for kids if your child sits still and can hold the Buzzy Bee for shots in place himself or you have another adult to help.

I wouldn’t say one works better than the other, but the ShotBlocker does have a couple of advantages. One, it’s cheaper. Second, it is easier to use when giving the injection by yourself. With one hand, you can hold the ShotBlocker and pinch the skin while giving the injection with the other hand. With the Buzzy, it’s easier to use if you have another person to hold it while you give the shot.

To learn more distraction techniques to use when giving painful shots, click here.

Click here for the Healthier Ever After injection tips booklet.

Click here for more tips and resources for patients on biologic medications for fever syndromes.