Background: Sarcoidosis is a multisystemic granulomatous disease with highly variable manifestations and outcomes, making clinical management challenging. This study evaluated the prognostic value of biomarkers derived from ¹⁸ F-FDG PET/CT imaging. Patients and Methods: From an institutional registry, 75 sarcoidosis patients with a ¹⁸ F-FDG PET/CT scan performed at the time of initial diagnosis were selected. A composite progression endpoint was defined, comprising either a decrease in FVC>5%, in DLCO>10%, a worsening of radiologic findings, increasing clinical symptoms, or death. Disease progression was assessed by onset at 1 year, 2 years, and overall. The MV of thoracic and extrathoracic lesions attributed to sarcoidosis was calculated, using a SUV threshold of 2.5. SUV _max was also determined for these regions. Results: Of 75 patients, 45 patients met the progression criteria while 30 patients remained in remission. Baseline MV _thoracic ( P = 0.002) and MV _whole-body ( P < 0.001) were higher in patients subsequently showing progression, while no significant differences were observed in whole-body or organ-specific SUV _max . The optimum threshold of MV _thoracic for prognosis of overall disease progression was determined at 150 cm ³ , yielding an OR of 1.9 in the univariate analysis (MV _thoracic >150 vs 150vs <150 cm ³ ; 95% CI: 1.08–3.95; P = 0.029). Neither total nor organ-specific SUVmax had significant associations with progression outcomes. Conclusions: Volume-based biomarkers derived from ¹⁸ F-FDG PET/CT were associated with disease progression in pulmonary sarcoidosis, whereas SUV _max showed no prognostic implications.

Background: The clinical spectrum of fibrotic interstitial lung diseases (ILDs) is highly heterogeneous. We aimed to evaluate the prognostic value of widely available baseline biomarkers for the improvement of lung function in patients with fibrotic ILDs. Methods: This registry-based study included 142 patients with fibrotic ILDs as defined by the presence of reticulation, traction bronchiectasis or honeycombing on initial high-resolution computed tomography (HRCT). Functional improvement at 1 year was defined as a relative increase of 5% in forced vital capacity (FVC) or of 10% in diffusion capacity for carbon monoxide (DLCO). The prognostic value of baseline biomarkers was evaluated for all patients and the subgroup with anti-inflammatory treatment. Results: At one year, 44 patients showed improvement while 73 showed disease progression. Multivariate analyses found prognostic significance for age < 60 years (OR 5.4; 95%CI 1.9–15.4; p = 0.002), lactate dehydrogenase (LDH) >250 U/L (OR 2.5; 95%CI 1.1–5.8; p = 0.043) and blood monocyte count < 0.8 G/L (OR 3.5; 95%CI 1.1–11.3; p = 0.034). In 84 patients undergoing anti-inflammatory treatment, multivariate analysis revealed age < 60 years (OR 8.5 (95%CI 2.1–33.4; p = 0.002) as the only significant variable. Conclusion: Younger age, a higher LDH and lower blood monocyte count predicted functional improvement in fibrotic ILD patients, while in those treated with anti-inflammatory drugs, only age had significant implications.

Zusammenfassung: Die Sarkoidose ist in vielen Fällen eine Multisystemerkrankung, die eine interdisziplinäre medizinische Zusammenarbeit in Diagnostik, Therapie und in der medizinischen Betreuung im Verlauf erfordert. Aufgrund des oft chronischen Verlaufes ist es besonders wichtig, Patientinnen und Patienten mit ihren Prioritäten und Wünschen frühzeitig und umfassend in die medizinische Betreuung einzubinden und, wenn möglich, ein „shared decision making“ zu etablieren. Beim Verfassen dieses gemeinsamen Positionspapieres war es der Expertengruppe für interstitielle Lungenerkrankungen und „orphan diseases“ der Österreichischen Gesellschaft für Pneumologie sowie der Arbeitsgruppe Rheuma und Lunge der Österreichischen Gesellschaft für Rheumatologie und Rehabilitation ein besonderes Anliegen, sowohl PatientInnenvertreter als auch ExpertInnen für seltenere Organmanifestationen der Sarkoidose einzubeziehen. Dieses Positionspapier soll nicht nur ein Spiegel der aktuellen klinischen und wissenschaftlichen Praxis sein, sondern auch die nationale Expertise bündeln und durch Vernetzung und Austausch ein erster Schritt zu einer Stärkung der Betreuungsstruktur von PatientInnen mit Sarkoidose sein.

Background: The clinical spectrum of fibrotic interstitial lung diseases (ILD) is highly heterogeneous. We aimed to evaluate the prognostic value of widely available baseline biomarkers for improvement of lung function in patients with fibrotic ILD. Methods: This registry-based study included 142 patients with fibrotic ILD as defined by presence of reticulation, traction bronchiectasis or honeycombing on initial high-resolution computed tomography (HRCT). Functional improvement at 1 year was defined as relative increase of 5% in forced vital capacity (FVC) or of 10% in diffusion capacity for carbon monoxide (DLCO). The prognostic value of baseline biomarkers was evaluated for all patients and the subgroup with anti-inflammatory treatment. Results: At one year, 44 patients showed improvement while 73 progressed. Multivariate analyses found prognostic significance for age 250U/L (OR 2.5; 95%CI 1.1-5.8; p=0.043) and blood monocyte count <0.8G/L (OR 3.5; 95%CI 1.1-11.3; p=0.034). In 84 patients undergoing anti-inflammatory treatment, multivariate analysis revealed age <60 years (OR 8.5 (95%CI 2.1-33.4; p=0.002) as the only significant variable. Conclusion: Younger age, higher LDH and lower blood monocyte count predicted functional improvement in fibrotic ILD patients, while in those treated with anti-inflammatory drugs, only age had significant implications.