Elisabeth Mulazzani1, Nicole Zolyniak2, Elisabeth Noe2, Matthias Mulazzani3, Shahnaz Christina Azad4, Tania Kümpfel5, Eduard Kraft2. 1. Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximillian University, Munich, Germany. elisabeth.schuh@med.uni-muenchen.de. 2. Department of Orthopaedics, Physical Medicine and Rehabilitation, Ludwig- Maximilians University, Munich, Germany. 3. Walter and Eliza Institute of Medical Research, Immunology Division, Melbourne, Australia. 4. Department of Anaesthesiology, Ludwig-Maximilian University, Munich, Germany. 5. Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximillian University, Munich, Germany.
Abstract
BACKGROUND: Pain is the clinical hallmark of patients in patients with autoinflammatory diseases (AID) caused by variants of the NLRP3-, MEFV- or TNFRSF1A gene. However, no systematical analysis of the clinical and psychological presentation of pain has been performed to date. METHODS: Twenty-one symptomatic patients with variants in the NLRP3-, MEFV- and TNFRSF1A gene and clinical signs suggestive of an AID were retrospectively included in this monocentric cross-sectional case-series study. Patients were examined and interviewed using the German pain questionnaire. The hospital anxiety and depression scale (HADS) was applied to screen patients for anxiety and depression. RESULTS: Twenty out of 21 AID patients (95%) reported pain at the time of examination. Mean current pain intensity in all AID patients comprised 3.6 ± 1.3 and mean maximum pain intensity was 7.0 ± 1.6 on a 11-point numeric ranging scale (NRS). In 15 patients (71%), pain was present for more than 60 months. Ten patients (48%) experienced recurrent attacks with asymptomatic intervals and 7 patients (33%) suffered from constant pain, while 4 patients (19%) experienced both. Nociceptive pain including musculoskeletal and visceral affection was the most prominent type of pain (n = 20; 95%). Pain symptoms were treated continuously with analgesic or co-analgesic drugs in 10 patients (48%). Five patients (24%) have been positively screened for concomitant depression or anxiety. CONCLUSIONS: Early and prompt diagnosis is necessary to provide multimodal pain treatment and to avoid the development of chronic pain in patients with AID.
BACKGROUND:Pain is the clinical hallmark of patients in patients with autoinflammatory diseases (AID) caused by variants of the NLRP3-, MEFV- or TNFRSF1A gene. However, no systematical analysis of the clinical and psychological presentation of pain has been performed to date. METHODS: Twenty-one symptomatic patients with variants in the NLRP3-, MEFV- and TNFRSF1A gene and clinical signs suggestive of an AID were retrospectively included in this monocentric cross-sectional case-series study. Patients were examined and interviewed using the German pain questionnaire. The hospital anxiety and depression scale (HADS) was applied to screen patients for anxiety and depression. RESULTS: Twenty out of 21 AID patients (95%) reported pain at the time of examination. Mean current pain intensity in all AID patients comprised 3.6 ± 1.3 and mean maximum pain intensity was 7.0 ± 1.6 on a 11-point numeric ranging scale (NRS). In 15 patients (71%), pain was present for more than 60 months. Ten patients (48%) experienced recurrent attacks with asymptomatic intervals and 7 patients (33%) suffered from constant pain, while 4 patients (19%) experienced both. Nociceptive pain including musculoskeletal and visceral affection was the most prominent type of pain (n = 20; 95%). Pain symptoms were treated continuously with analgesic or co-analgesic drugs in 10 patients (48%). Five patients (24%) have been positively screened for concomitant depression or anxiety. CONCLUSIONS: Early and prompt diagnosis is necessary to provide multimodal pain treatment and to avoid the development of chronic pain in patients with AID.
Authors: Philip L De Jager; Xiaoming Jia; Joanne Wang; Paul I W de Bakker; Linda Ottoboni; Neelum T Aggarwal; Laura Piccio; Soumya Raychaudhuri; Dong Tran; Cristin Aubin; Rebeccah Briskin; Susan Romano; Sergio E Baranzini; Jacob L McCauley; Margaret A Pericak-Vance; Jonathan L Haines; Rachel A Gibson; Yvonne Naeglin; Bernard Uitdehaag; Paul M Matthews; Ludwig Kappos; Chris Polman; Wendy L McArdle; David P Strachan; Denis Evans; Anne H Cross; Mark J Daly; Alastair Compston; Stephen J Sawcer; Howard L Weiner; Stephen L Hauser; David A Hafler; Jorge R Oksenberg Journal: Nat Genet Date: 2009-06-14 Impact factor: 38.330