Risk factors for cystic fibrosis arthropathy: Data from the German cystic fibrosis registry

Grehn, Claudia; Dittrich, Anna-Maria; Wosniok, J.; Holz, F.; Hafkemeyer, Sylvia; Naehrlich, Lutz; Schwarz, C.

doi:10.1016/j.jcf.2021.05.003

Cited by 7 publications

(8 citation statements)

References 38 publications

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“…We also reported that patients suffering from spinal and/or joint pain had significantly more frequent F508 homozygous mutations (59 vs. 24%, p = 0.014). By contrast, two previous studies didn't find an association between cystic fibrosis transmembrane conductance regulator (CFTR) mutations and CFA (7,9).…”

Section: Discussionmentioning

confidence: 76%

“…The comparison with other studies is difficult in the absence of an accepted definition of CFA and with various study designs. These studies described a prevalence of inflammatory joint included between 2 and 29% of the patients ( 6 – 9 , 28 ). In Koch et al study there was no significant difference regarding inflammatory signs such as joint swelling or warming between CF patients and controls ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

“…and pulmonary exacerbations ( 7 ). In Grehn et al study from the German CF registry, CFA including arthropathy and arthritis was associated with increasing age, female gender, number of hospitalizations, chronic Pseudomonas aeruginosa infection, CF-related diabetes, pancreatic insufficiency and sinusitis/polyps ( 9 ). These results also support a correlation between pulmonary inflammation/infection and CFA.…”

Section: Discussionmentioning

confidence: 99%

“…With improving survival, the prevalence of long-term complications increased (3). In CF adults, rheumatologic disorders are frequently encountered (4,5) including CF arthropathy (CFA) (2-29%) (6)(7)(8)(9), CF related bone disease (CFBD) including osteoporosis, fractures, and musculoskeletal manifestations (13 to 35%) (10)(11)(12)(13), hypertrophic pulmonary osteoarthropathy (2 to 7%) and quinolone-induced arthropathy (14).…”

Section: Introductionmentioning

confidence: 99%

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Prevalence and Impact of Rheumatologic Pain in Cystic Fibrosis Adult Patients

et al. 2022

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BackgroundWith the improvement of cystic fibrosis (CF) patient survival, the prevalence of long-term complications increased, among them rheumatologic disorders.MethodsThe aim of this prospective study was to evaluate the prevalence of spinal and joint pain, and their impact on disability, anxiety, depression, and quality of life in CF adult patients.ResultsForty-seven patients were analyzed, 72% of men, mean aged 28 years, with a mean body mass index of 22 kg/m2 and a mean FEV1% of 63%. Twenty-two patients (47%) described rheumatologic pain either spinal (n = 15, 32%) and/or joint pain (n = 14, 30%). Patients with spinal and/or joint pain were shorter (p = 0.023), more frequently colonized with Staphylococcus aureus (p < 0.008), had more frequent ΔF508 homozygous mutations (p = 0.014), and a trend for more impairment of the 6-min walking distance (p = 0.050). The presence of rheumatologic pain tended to be associated with disability according to the Health Assessment Questionnaire (HAQ) and anxiety. Compared with patients with no pain patients with both spinal and joint pain exhibited a more pronounced impact on the St George's Respiratory Questionnaire (SGRQ).ConclusionRheumatologic pain is frequent in CF adult patients, and may affect daily living, anxiety and quality of life. Systematic assessment of rheumatologic pain should be included in the management of CF patients.

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Section: Discussionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Prevalence and Impact of Rheumatologic Pain in Cystic Fibrosis Adult Patients

et al. 2022

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“…For patients with cystic fibrosis (CF), less information is available in field literature, and some studies point to an overall lower incidence of SARS-CoV-2 infection in this patient population [ 1 , 2 ], but a higher rate of hospitalization among them [ 3 ]. Furthermore, once infection does occur, the patient is considered to be at risk of severe COVID-19 and this risk may be dependent on the underlying degree of lung damage, and also the degree of pancreatic or liver damage [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

First Case of COVID-19 Treated with Monoclonal Anti-Spike Antibodies in a Patient with Cystic Fibrosis in Romania

et al. 2022

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Patients with chronic lung conditions, including cystic fibrosis, may be prone to severe COVID-19. Therefore, therapeutic intervention should be prompt and tailored to all associated comorbidities. We report the case of a 17-year-old male adolescent with cystic fibrosis and multiple chronic conditions (bronchiectasis, exocrine pancreatic insufficiency, chronic multidrug resistant Pseudomonas aeruginosa colonization, nasal polyposis, chronic sinusitis, ventricular extrasystoles and multiple drug allergies), who presented with an acute episode of productive cough, and was confirmed with moderate COVID-19 based on positive RT-PCR for SARS-CoV-2 and lung imaging showing isolated foci of interstitial pneumonia. Intravenous treatment with the monoclonal antibody cocktail casirivimab and imdevimab was administered. The evolution was favorable, with rapid remission of the inflammatory syndrome and gradual decrease of cough, without progression to severe or critical COVID-19, but with complications such as repeated hemoptysis, which was due to the patient’s underlying conditions, and which required close monitoring for timely adjustment of the patient’s chronic treatment.

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