Clinical and psychological phenomenology of pain in autoinflammatory diseases
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Mulazzani et al. BMC Rheumatology (2020) 4:71 https://doi.org/10.1186/s41927-020-00168-x
RESEARCH ARTICLE
Open Access
Clinical and psychological phenomenology of pain in autoinflammatory diseases Elisabeth Mulazzani1*, Nicole Zolyniak2, Elisabeth Noe2, Matthias Mulazzani3, Shahnaz Christina Azad4, Tania Kümpfel1 and Eduard Kraft2
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. Keywords: Pain, Autoinflammatory diseases, Low penetrance variants
Background Pain is one of the predominant characteristics in patients with autoinflammatory diseases (AID), including the cryopyrin-associated periodic syndrome (CAPS), familial Mediterranean fever (FMF) or tumor necrosis factor receptor associated periodic syndrome (TRAPS). It is also the experience of pain that significantly impacts patient’s daily life activities and drives patients with autoinflammatory
* Correspondence: [email protected] 1 Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximillian University, Munich, Germany Full list of author information is available at the end of the article
syndromes to seek medical care and to use non-steroidal anti-inflammatory drugs [1]. Although the condition of pain is a diagnostic criterion in AID, only few data on clinical and psychological pain characteristics exist. The clinical picture of AID are commonly characterized by painful flares of systemic inflammation in joints, skin, muscles, eyes and serosal surfac
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