Objectives: To describe the characteristics and progression of interstitial lung disease in patients with associated systemic autoimmune disease (ILD-SAI) and to identify factors associated with ...progression and mortality. Patients and methods: We performed a multicenter, retrospective, observational study of patients with ILD-SAI followed between 2015 and 2020. We collected clinical data and performed pulmonary function testing and high-resolution computed tomography at diagnosis and at the final visit. The main outcome measure at the end of follow-up was forced vital capacity (FVC) >10% or diffusing capacity of the lungs for carbon monoxide >15% and radiological progression or death. Cox regression analysis was performed to identify factors associated with worsening of ILD. Results: We included 204 patients with ILD-SAI: 123 (60.3%) had rheumatoid arthritis (RA), 58 had (28.4%) systemic sclerosis, and 23 (11.3%) had inflammatory myopathy. After a median (IQR) period of 56 (29.8–93.3) months, lung disease had stabilized in 98 patients (48%), improved in 33 (16.1%), and worsened in 44 (21.5%). A total of 29 patients (14.2%) died. Progression and hospitalization were more frequent in patients with RA (p = 0.010). The multivariate analysis showed the independent predictors for worsening of ILD-SAI to be RA (HR, 1.9 95% CI, 1.3–2.7), usual interstitial pneumonia pattern (HR, 1.7 95% CI, 1.0–2.9), FVC (%) (HR, 2.3 95% CI, 1.4–3.9), and smoking (HR, 2.7 95%CI, 1.6–4.7). Conclusion: Disease stabilizes or improves after a median of 5 years in more than half of patients with ILD-SAI, although more than one-third die. Data on subgroups and risk factors could help us to predict poorer outcomes.
To describe severe infection, foci of infection, microorganisms, associated factors, and impact on mortality in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD).
The ...study was based on a multicenter prospective cohort of patients with RA-ILD followed up from 2015 to 2023. The main outcome measures were incident severe infection and fatal infection. We evaluated infectious foci, etiologic agents, vaccination status, variables associated with lung function, and clinical-therapeutic variables in RA. The incidence rate (IR) for infection and mortality was calculated per 100 person-years, and 3 multivariate models were constructed to explore factors associated with infection.
We followed up 148 patients with RA-ILD for a median 56.7 months (699.3 person-years). During this period, 142 patients (96%) had at least 1 infection. A total of 368 infectious episodes were recorded, with an IR of 52.6 per 100 person-years. Of the 48 patients who died, 65% did so from infection. Respiratory infections were the most common first infection (74%), infection overall (74%), and fatal infection (80%) and were caused mostly by SARS CoV-2
, and influenza A virus. The factors associated with an increased risk of infection and death in patients with RA-ILD were age, inflammatory activity, and therapy with corticosteroids and immunosuppressants.
Patients with RA-ILD have a high risk of serious infection, especially respiratory infection. Infection develops early, is recurrent, and is frequently fatal. The presence of associated factors such as advanced age, joint inflammation, and treatment highlight the importance of integrated and preventive medical care.
To analyze the efficacy and safety of rituximab (RTX) in connective tissue disease associated with interstitial lung disease (CTD-ILD).
We performed a multicenter, prospective, observational study of ...patients with CTD-ILD receiving rituximab between 2015 and 2020. The patients were assessed using high-resolution computed tomography and pulmonary function tests at baseline, at 12 months, and at the end of follow-up. The main outcome measure at the end of follow-up was forced vital capacity (FVC) > 10% or diffusing capacity of the lungs for carbon monoxide (DLCO) > 15% and radiological progression or death. We recorded clinical characteristics, time to initiation of RTX, concomitant treatment, infections, and hospitalization. A Cox regression analysis was performed to identify factors associated with worsening ILD.
We included 37 patients with CTD-ILD treated with RTX for a median (IQR) of 38.2 (17.7-69.0) months. At the end of the follow-up, disease had improved or stabilized in 23 patients (62.1%) and worsened in seven (18.9%); seven patients (18.9%) died. No significant decline was observed in median FVC (72.2 vs. 70.8;
= 0.530) or DLCO (55.9 vs. 52.2;
= 0.100). The multivariate analysis showed the independent predictors for worsening of CTD-ILD to be baseline DLCO (OR (95% CI), 0.904 (0.8-0.9);
= 0.015), time to initiation of RTX (1.01 (1.001-1.02);
= 0.029), and mycophenolate (0.202 (0.04-0.8);
= 0.034). Only 28 of the 37 patients (75.6%) were still undergoing treatment with RTX: two patients (5.4%) stopped treatment due to adverse events and seven patients (18.9%) died owing to progression of ILD and superinfection.
Lung function improved or stabilized in more than half of patients with CTD-ILD treated with RTX. Early treatment and combination with mycophenolate could reduce the risk of progression of ILD.
Objective: To prospectively evaluate the safety and efficacy profile of abatacept in patients with rheumatoid arthritis–associated interstitial lung disease (RA-ILD). Methods: We performed a ...prospective observational multicenter study of a cohort of patients with RA-ILD treated with abatacept between 2015 and 2021. Patients were evaluated using high-resolution computed tomography and pulmonary function tests at initiation, 12 months, and the end of follow-up. The effectiveness of abatacept was evaluated based on whether ILD improved, stabilized, progressed, or was fatal. We also evaluated factors such as infection, hospitalization, and inflammatory activity using the 28-joint Disease Activity Score with the erythrocyte sedimentation rate (DAS28-ESR). Cox regression analysis was performed to identify factors associated with progression of lung disease. Results: The study population comprised 57 patients with RA-ILD treated with abatacept for a median (IQR) of 27.3 (12.2–42.8) months. Lung disease had progressed before starting abatacept in 45.6% of patients. At the end of follow-up, lung disease had improved or stabilized in 41 patients (71.9%) and worsened in 13 (22.8%); 3 patients (5.3%) died. No significant decreases were observed in forced vital capacity (FVC) or in the diffusing capacity of the lung for carbon monoxide (DLCO).The factors associated with progression of RA-ILD were baseline DAS28-ESR (OR 95% CI, 2.52 1.03–3.12; p = 0.041), FVC (OR 95% CI, 0.82 0.70–0.96; p = 0.019), and DLCO (OR 95% CI, 0.83 0.72–0.96; p = 0.018). Only 10.5% of patients experienced severe adverse effects. Conclusion: Pulmonary function and joint inflammation stabilized in 71% of patients with RA-ILD treated with abatacept. Abatacept had a favorable safety profile.
Neurosarcoidosis is a rare complication of sarcoidosis. There are small series on the condition and very few from Spain. We conducted a retrospective study of neurosarcoidosis in Virgen de la ...Victoria Hospital over the last 10 years.
the medical records of patients diagnosed with sarcoidosis in our setting in the last 10 years were reviewed.
One hundred twenty patients with sarcoidosis, 20 patients with probable neurosarcoidosis: 30% at the beginning of the illness, 55% later, 15% had isolated neurosarcoidosis. Forty percent had polyneuropathy, 15% cranial neuropathy, 15% myopathy, 10% hemispheric symptoms. OTHERS: headache, myelitis, seizures, confusional syndrome. They were treated with steroids, some of them with immunosuppressive treatment. Seventy-two percent improved or were stabilized. Therefore, neurosarcoidosis prevalence in our hospital was 11%, similar to other published series, with similar features, but polyneuropathy was more frequent. Early diagnosis is very important as prognosis is favourable with treatment.
Neurosarcoidosis is a rare complication of sarcoidosis. There are small series on the condition and very few from Spain. We conducted a retrospective study of neurosarcoidosis in Virgen de la ...Victoria Hospital over the last 10 years.
the medical records of patients diagnosed with sarcoidosis in our setting in the last 10 years were reviewed.
120 patients with sarcoidosis, 20 patients with probable neurosarcoidosis: 30% at the beginning of the illness, 55% later, 15% had isolated neurosarcoidosis. Forty percent had polyneuropathy, 15% cranial neuropathy, 15% myopathy, 10% hemispheric symptoms.
Headache, myelitis, seizures, confusional syndrome. They were treated with steroids, some of them with immunosuppressive treatment. Seventy-two percent improved or were stabilized. Therefore, neurosarcoidosis prevalence in our hospital was 11%, similar to other published series, with similar features, but polyneuropathy was more frequent. Early diagnosis is very important as prognosis is favourable with treatment.
La neurosarcoidosis es una rara complicación de la sarcoidosis. Existen series cortas de pacientes con esta afectación. En España son prácticamente inexistentes. Planteamos realizar un estudio retrospectivo sobre neurosarcoidosis en el hospital Virgen de la Victoria a lo largo de diez años.
Búsqueda en la base de datos hospitalaria de los pacientes con este diagnóstico en los últimos diez años.
120 pacientes con sarcoidosis, de los cuales, 20 pacientes compatibles con neurosarcoidosis probable: 30% fue debut de la enfermedad, 55% ya estaban diagnosticados de sarcoidosis, y 15% tuvieron neurosarcoidosis aislada. El 40% presentaron polineuropatía, 15% neuropatía craneal, 15% miopatía, 10% focalidad SNC.
Cefalea, mielitis, crisis epiléptica, cuadro confusional. Se trataron con esteroides, y a veces asociaron otros inmunosupresores. El 72% de los casos se estabilizaron o hubo mejoría. Por tanto, la prevalencia de neurosarcoidosis en nuestro hospital es del 11%, parecido a otras series publicadas, con características similares, pero con mayor frecuencia de polineuropatía. El pronóstico de la enfermedad es aceptablemente favorable con tratamiento, por lo que es importante el diagnóstico precoz.
Objectives: To describe the frequency of COVID-19 and the effect of vaccination in patients with interstitial lung disease and systemic autoimmune disease (ILD-SAD) and to identify factors associated ...with infection and severity of COVID-19. Methods: We performed a cross-sectional multicenter study of patients with ILD-SAD followed between June and October 2021. The main variable was COVID-19 infection confirmed by a positive polymerase chain reaction (PCR) result for SARS-CoV-2. The secondary variables included severity of COVID-19, if the patient had to be admitted to hospital or died of the disease, and vaccination status. Other variables included clinical and treatment characteristics, pulmonary function and high-resolution computed tomography. Two logistic regression was performed to explore factors associated with “COVID-19” and “severe COVID-19”. Results: We included 176 patients with ILD-SAD: 105 (59.7%) had rheumatoid arthritis, 49 (27.8%) systemic sclerosis, and 22 (12.54%) inflammatory myopathies. We recorded 22/179 (12.5%) SARS-CoV-2 infections, 7/22 (31.8%) of them were severe and 3/22 (13.22%) died. As to the vaccination, 163/176 (92.6%) patients received the complete doses. The factors associated with SARS-CoV-2 infection were FVC (OR (95% CI), 0.971 (0.946−0.989); p = 0.040), vaccination (OR (95% CI), 0.169 (0.030−0.570); p = 0.004), and rituximab (OR (95% CI), 3.490 (1.129−6.100); p = 0.029). The factors associated with severe COVID-19 were the protective effect of the vaccine (OR (95% CI), 0.024 (0.004−0.170); p < 0.001) and diabetes mellitus (OR (95% CI), 4.923 (1.508−19.097); p = 0.018). Conclusions: Around 13% of patients with ILD-SAD had SARS-CoV-2 infection, which was severe in approximately one-third. Most patients with severe infection were not fully vaccinated.
To describe comorbid conditions in patients with rheumatoid arthritis–associated interstitial lung disease (RA-ILD) and to analyze factors associated with multimorbidity.
Nested case-cohort study of ...2 prospective cohorts: one with RA-ILD (cases) and another with RA but not ILD (controls). The cohorts were matched for age, sex, and time since diagnosis. Multimorbidity was defined as the co-occurrence of 2 or more chronic diseases, in addition to RA and ILD. We evaluated the comorbid conditions included in the Charlson Comorbidity Index, cardiovascular risk factors, neuropsychiatric conditions, and other frequent conditions in RA. We also recorded clinical-laboratory variables, inflammatory activity according to the 28-joint Disease Activity Score, C-reactive protein (CRP), physical function, and pulmonary function. We performed 2 multivariate analyses to identify factors associated with multimorbidity in RA and RA-ILD.
The final study population comprised 110 cases and 104 controls. Multimorbidity was more frequent among cases than controls (80 72.7 vs 60 57.7; p = 0.021). In both groups, multimorbidity was associated with ILD (OR 95% CI 1.92 1.03–3.59; p = 0.039), age (OR 95% CI 1.05 1.01–1.08; p = 0.004), CRP (OR 95% CI 1.16 1.05–1.29; p = 0.003), and erosions (OR 95% CI 1.05 1.01–1.08; p = 0.004); in the cases, it was associated with CRP (OR 95% CI 1.17 1.01–1.35; p = 0.027), anti–citrullinated peptide antibody (OR 95% CI 1.23 1.14–13.02; p = 0.049), and forced vital capacity (OR 95% CI 0.79 0.96–0.99; p = 0.036).
In patients with RA, multimorbidity was associated with ILD, systemic inflammation, and advanced age.
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•Comorbid conditions are a major cause of morbidity and mortality in patients with rheumatoid arthritis (RA) and patients with interstitial lung disease (ILD).•ILD was independently associated with multimorbidity in patients with RA.•The most frequent comorbid conditions associated with RA-ILD were traditional cardiovascular risk factors, depression, and osteoporosis.•Other factors, such as ACPA titers and elevated CRP levels, were also associated with multimorbidity in cases with RA-ILD.
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