…due to improved understanding and treatment of their condition. Rarity of HIDS HIDS is one of the rarer autoinflammatory conditions. As of 2014, estimates show there are about 300 total confirmed HIDS cases in the world. Most are found in The Netherlands. Less than 100 HIDS patients have been identified by researchers in the U.S. Likely this is an under-diagnosed autoinflammatory disease, in part because the symptoms can very closely resemble PFA…
…ation varies among patients. Not every patient has every symptom. PFAPA vs HIDS PFAPA HIDS Symptoms Fever accompanied by pharyngitis and at least one of the following symptoms for a diagnosis: mouth ulcers, and/or swollen neck lymph nodes. Fever with several other inflammatory symptoms. These can include: mouth ulcers, pharyngitis, swollen lymph nodes, joint swelling and pain, abdominal pain, diarrhea, vomiting, headaches, enlarged liver, and/or…
…elevated IgD is not diagnostic to HIDS. In 1999, the gene responsible for HIDS was found. HIDS and MA (mevalonate aciduria) are caused by mutations in the mevalonate kinase gene (MVK), giving rise to the new classification for these conditions–mevalonate kinase deficiencies (MKD). This discovery led to great advances in understanding HIDS and MA. By using genetic testing as part of the diagnosis and research into HIDS, studies now show that eleva…