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Boston – Autoinflammatory and autoimmune disorders in children have many overlapping features, but the former represents a malfunction of the innate immune system, and the latter a malfunction of the adaptive immune system. Fevers, skin eruptions, joint pain and swelling, fatigue, and growth failure can occur in both, but it is important to identify the underlying problem, according to Raegan Hunt, MD.
Autoinflammatory disorders can be difficult to detect, so it is important to maintain a high index of suspicion for them, Dr. Hunt of Baylor College of Medicine and Texas Children’s Hospital, Houston, said at the American Academy of Dermatology annual summer meeting.
She discussed five signs of possible autoinflammatory disease in children, as described in a recent report by Hal M. Hoffman, MD, and Lori Broderick, MD, of Children’s Hospital of San Diego, La Jolla, Calif. (J Allergy Clin Immunol. July 2016; 138:3-14)*:
1. More than three episodes of fever over 101 degrees Farenheit with no explained infectious etiology.
2. Having predictable patterns of characteristic course of each episode.
3. Having specific symptoms during episodes, including nonpruritic skin eruptions, joint or bone pain, severe abdominal pain, and conjunctivitis, with no upper respiratory infection symptoms.
4. Episodes may be triggered by specific stimuli, such as cold exposure or vaccines.
5. A family history of autoinflammatory disease or amyloidosis.
The authors “propose that two or more of these might suggest that an autoinflammatory disorder is possible and should be investigated,” Dr. Hunt said.
Dr. Hunt reported having no relevant disclosures.
*Correction, 8/16/16: An earlier version of this article failed to note that Dr. Lori Broderick was a co-investigator in the cited report.
Boston – Autoinflammatory and autoimmune disorders in children have many overlapping features, but the former represents a malfunction of the innate immune system, and the latter a malfunction of the adaptive immune system. Fevers, skin eruptions, joint pain and swelling, fatigue, and growth failure can occur in both, but it is important to identify the underlying problem, according to Raegan Hunt, MD.
Autoinflammatory disorders can be difficult to detect, so it is important to maintain a high index of suspicion for them, Dr. Hunt of Baylor College of Medicine and Texas Children’s Hospital, Houston, said at the American Academy of Dermatology annual summer meeting.
She discussed five signs of possible autoinflammatory disease in children, as described in a recent report by Hal M. Hoffman, MD, and Lori Broderick, MD, of Children’s Hospital of San Diego, La Jolla, Calif. (J Allergy Clin Immunol. July 2016; 138:3-14)*:
1. More than three episodes of fever over 101 degrees Farenheit with no explained infectious etiology.
2. Having predictable patterns of characteristic course of each episode.
3. Having specific symptoms during episodes, including nonpruritic skin eruptions, joint or bone pain, severe abdominal pain, and conjunctivitis, with no upper respiratory infection symptoms.
4. Episodes may be triggered by specific stimuli, such as cold exposure or vaccines.
5. A family history of autoinflammatory disease or amyloidosis.
The authors “propose that two or more of these might suggest that an autoinflammatory disorder is possible and should be investigated,” Dr. Hunt said.
Dr. Hunt reported having no relevant disclosures.
*Correction, 8/16/16: An earlier version of this article failed to note that Dr. Lori Broderick was a co-investigator in the cited report.
Boston – Autoinflammatory and autoimmune disorders in children have many overlapping features, but the former represents a malfunction of the innate immune system, and the latter a malfunction of the adaptive immune system. Fevers, skin eruptions, joint pain and swelling, fatigue, and growth failure can occur in both, but it is important to identify the underlying problem, according to Raegan Hunt, MD.
Autoinflammatory disorders can be difficult to detect, so it is important to maintain a high index of suspicion for them, Dr. Hunt of Baylor College of Medicine and Texas Children’s Hospital, Houston, said at the American Academy of Dermatology annual summer meeting.
She discussed five signs of possible autoinflammatory disease in children, as described in a recent report by Hal M. Hoffman, MD, and Lori Broderick, MD, of Children’s Hospital of San Diego, La Jolla, Calif. (J Allergy Clin Immunol. July 2016; 138:3-14)*:
1. More than three episodes of fever over 101 degrees Farenheit with no explained infectious etiology.
2. Having predictable patterns of characteristic course of each episode.
3. Having specific symptoms during episodes, including nonpruritic skin eruptions, joint or bone pain, severe abdominal pain, and conjunctivitis, with no upper respiratory infection symptoms.
4. Episodes may be triggered by specific stimuli, such as cold exposure or vaccines.
5. A family history of autoinflammatory disease or amyloidosis.
The authors “propose that two or more of these might suggest that an autoinflammatory disorder is possible and should be investigated,” Dr. Hunt said.
Dr. Hunt reported having no relevant disclosures.
*Correction, 8/16/16: An earlier version of this article failed to note that Dr. Lori Broderick was a co-investigator in the cited report.
Expert Analysis from the AAD Summer Academy 2016