arthritis mutilans
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2021 ◽  
Vol 8 ◽  
Josephine Mistegård ◽  
Bjorn Gudbjornsson ◽  
Ulla Lindqvist ◽  
Leena Laasonen ◽  
Leif Ejstrup ◽  

Objective: Psoriatic arthritis mutilans (PAM) is the most severe phenotype of psoriatic arthritis due to excessive bone erosion causing joint destruction and decreased functional capacity. The aim of this study was to investigate the prevalence of comorbidities among patients with PAM and the association between comorbidities and joint involvement.Methods: A total of 66 patients aged ≥18 years from the Nordic countries with past or present psoriasis along with at least one mutilated joint were included in the present study.Results: The median number of comorbid conditions per patient was 1 [interquartile range (IQR) 0–2] and 16.7% reported three or more comorbidities. The most frequent comorbidity was hypertension (36.4%). The median number of mutilated joints per patient was 3 (IQR 1–8.3; range 1–38).Conclusion: Two thirds of the patients with PAM reported comorbid conditions and the most frequent was hypertension which affected more than a third of the patients. However, this study was unable to detect any association between comorbidities and the severity of PAM.

Takako Miyamae ◽  
Midori Akatsu ◽  
Naomi Ichikawa ◽  
Atsuo Taniguchi ◽  
Masayashi Harigai

2020 ◽  
Vol 4 (Supplement_1) ◽  
Ritsuko Saito ◽  
Ernest Wong

Abstract Case report - Introduction Granulomatous disorders are diverse in their aetiologies and presentations. We present an unusual case of severe psoriatic arthritis patient who subsequently developed multiple granulomatous diseases over time, granulomatous interstitial nephritis, granulomatous sarcoidosis with hilar lymphadenopathy and localised laryngeal granulomatous inflammation secondary to lambda type amyloidosis. Case report - Case description 52-year-old gentleman with arthritis mutilans secondary to severe poorly controlled psoriatic arthritis was followed up in Rheumatology clinic. Earlier therapy with leflunomide and methotrexate provided inadequate control. Golimumab, despite giving a good response, was stopped in 2013 after 5 months of treatment due to acute kidney injury. Renal biopsy revealed granulomatous interstitial nephritis, thought to be Golimumab-induced based on the timing of usage and reversibility with discontinuation. He was then trialled on Ustekinumab and Secukinumab in 2016 and 2017 respectively with variable response. He also had a few-years history of voice change (high pitched) and sore throat which he attributed to recurrent colds. He denied dysphagia or breathlessness, and he did not have stridor. He has never smoked and only drank alcohol occasionally. ENT team noted white deposits on erythematous and thickened false vocal cords and posterior glottis with a thin web on microlaryngoscopy, which histologically proved to show granulomatous inflammation, potentially consisting of amyloid, although congo red stain was negative. On further investigation, including SAP scan, he was diagnosed with localised lambda type amyloid. Increasing throat pain and worsening dysphonia prompted change of management from conservative to a surgery at a specialist centre and an input from speech and language therapy team. During this time, consideration for Etanercept for his joint and skin disease was put on hold, pending further management of laryngeal amyloidosis. Furthermore, he presented to hospital with breathlessness in 2019, where his chest X-ray showed bulky right hilum and a follow-up CT chest revealed calcified right hilar and mediastinal lymphadenopathy, ground glass opacification and consolidation. Histology from hilar node was suggestive of sarcoidosis, with stain negative for amyloid. He underwent removal of false vocal cords for his symptomatic laryngeal amyloidosis. He continues to be followed up at the local Rheumatology, Dermatology and ENT team. Case report - Discussion Granulomatous diseases have vast aetiologies, including infectious, immunological, neoplastic, and chemical-induced processes. The age at which they affect patients and tissue they involve also vary hugely. This is the first reported case of three seemingly unrelated granulomatous diseases occurring in a single patient with severe refractory psoriatic arthritis. Retrospective reassessment of the histology samples supported that these are three separate pathologies. It is very unusual for one patient to acquire multiple separate granulomatous diseases, which was why the diagnostic process of this patient was challenging. In this case, managing the original underlying psoriatic arthritis was particularly difficult due to interruptions of treatment for adverse drug effects and investigations and treatment of subsequent granulomatous diseases. The case also raises questions about possible currently unknown association between the pathologies. Case report - Key learning points Key points are the uniqueness of this case and that it highlighted the possibility of currently under-reported association between these three granulomatous conditions. As ever, a multidisciplinary approach to managing such a complex patient is important for the provision of good care.

2020 ◽  
Vol 4 (1) ◽  
pp. 002-002
Mullick Gautam ◽  
Handa Ajay ◽  
Chauhan Vikas

2020 ◽  
Henry Knipe

2020 ◽  
Vol 9 (4) ◽  
pp. 205846012092079
Leena Laasonen ◽  
Ulla Lindqvist ◽  
Lars Iversen ◽  
Leif Ejstrup ◽  
Thorarinn Jonmundsson ◽  

Background Psoriatic arthritis mutilans (PAM) is the most severe phenotype of psoriatic arthritis (PsA). Purpose To describe the radiological features in PAM and explore whether existing scoring systems for radiological damage in psoriatic arthritis are applicable for PAM. Material and Methods Radiographs were scored according to the modified Sharp-van der Heijde (mSvdH) and the Psoriatic Arthritis Ratingen Score (PARS) systems for PsA. Results At inclusion, 55 PAM patients (49% women, mean age 58 ± 12 years) had conventional radiographs of both hands and feet. A total of 869 PAM joints were detected and 193 joints with ankylosis. The mean total mSvdH score was 213.7 ± 137.8 (41% of maximum) with a higher score for hands than for feet: 136.6 ± 90.1 vs. 79.1 ± 60.9. However, the total score was relatively higher in the feet than in the hands when compared to the highest possible scoring (47% vs. 38% of max). The mean total PARS score was 126.3 ± 79.6 (35% of max). Scoring for joint destruction was higher than for proliferation (22% vs. 11% of max). Strong correlation was found between mSvdH and PARS (r2 = 0.913). A significant correlation was found between scoring and duration of arthritis and the Health Assessment Questionnaire. History of smoking, BMI, and gender did not influence the scoring values. Conclusions The two scoring systems studied may not be ideal to indicate progression of PAM in advanced disease since they reach ceiling effects rather early. Therefore, reporting early signs suggestive of PAM, e.g. signs of pencil-in-cup deformities or osteolysis, is crucial. This would reveal the presence of PAM and might lead to improved treatment in order to minimize joint damage.

QJM ◽  
2020 ◽  
Vol 113 (10) ◽  
pp. 765-766
J Samanta ◽  
A Chattopadhyay ◽  
A Sharma

Reumatismo ◽  
2019 ◽  
Vol 71 (2) ◽  
pp. 88-91
F.M. Perrotta ◽  
S. Scriffignano ◽  
A. De Socio ◽  
E. Lubrano

We present the case of a patient with psoriatic arthritis (PsA) mutilans and occult axial involvement. The patient had suffered from PsA mutilans for more than a decade, with severe residual articular damage, but had been in clinical remission for years. Clinical axial involvement was never reported; however, magnetic resonance imaging of the sacroiliac joints, performed for other reasons, documented active inflammation and damage even without clinical symptoms. These findings corroborated the hypothesis that axial involvement could be asymptomatic, subclinical and, furthermore, underdiagnosed or even occult in patients with PsA mutilans, in which cases it should be carefully evaluated.

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