Propionibacterium acnes-associated sarcoidosis complicated by acute bird-related hypersensitivity pneumonitis.

BACKGROUND: The number of reports on sarcoidosis complicated by hypersensitivity pneumonitis (HP) is limited, and most describe cases complicated by chronic bird-related HP. Here, we present for the first time a case with Propionibacterium acnes-associated sarcoidosis complicated by acute bird-related HP. CASE PRESENTATION: A 62-year-old man with a past medical history of sarcoidosis was admitted to our department, and chest computed tomography showed diffuse ground-glass opacities, which appeared as he rapidly increased the number of pigeons he kept for a competition. Random transbronchial lung biopsy revealed well-formed non-caseating epithelioid granulomas, which contained positively stained substances on immunohistochemistry using the PAB antibody, a specific monoclonal antibody against P. acnes lipoteichoic acid. Poorly formed non-caseating granulomas without positively stained substances were also detected. CONCLUSION: We describe the successful identification of this exceptionally rare case of sarcoidosis complicated by acute bird-related HP in which two morphologically and immunohistologically different types of granulomas were present in the same lung.

Central Bronchial Deformity in Pulmonary Sarcoidosis: A Finding Suggestive of an Upper Lobe Fibrotic Phenotype on Chest Images.

RATIONALE AND OBJECTIVES: Bronchial and lung parenchymal structural remodeling may occur due to disease progression in patients with pulmonary sarcoidosis; however, its mechanisms remain unclear. Central bronchial deformity (CBD) associated with shrinkage in the upper lobe (SUL) is often observed in such patients. This study aimed to examine the association between CBD and structural remodeling to identify features indicating disease severity on chest images. MATERIALS AND METHODS: This retrospective cohort study included 72 patients with pulmonary sarcoidosis, excluding patients with only bilateral hilar lymphadenopathy. The participants were divided into with and without CBD groups to examine the association between CBD and other structural remodeling, including SUL, cyst and/or low attenuation area-like emphysema (Cyst/LAA), pleural/sub-pleural thickening (PT), and traction bronchiectasis (TrBE), in the upper lobe on chest images. The association of CBD phenotype with respiratory dysfunction was also examined. RESULTS: CBD was highly associated with SUL (81.4% vs. 8.9%), Cyst/LAA (44.4% vs. 6.7%), and PT (59.2% vs. 3.7%). The respective odds ratios in the univariable and multivariable analyses were as follows: SUL, 45.1 and 39.9; Cyst/LAA, 11.2 and 14.2; and PT, 64.0 and 68.7. TrBE was frequently associated with CBD (22.25% vs. 4.4%); the odds ratio was 6.14 in the univariable analysis. Furthermore, participants with CBD exhibited lower %FVC and %DLCO. CONCLUSION: CBD is significantly associated with lung remodeling (SUL, Cyst/LAA, TrBE, and PT) and respiratory dysfunction. CBD may be a crucial clinical phenotype to identify upper lobe fibrotic changes.

Prevalence of systemic and local risk factors for pulmonary non-tuberculous mycobacterial disease in Japan: a single-institution study.

Objective: To identify the prevalence of risk factors for pulmonary non-tuberculous mycobacterial (NTM) disease in a Japanese population. Patients and Methods: We reviewed 337 consecutive Japanese patients (210 women) with pulmonary NTM disease, including 225 patients with Mycobacterium avium complex (MAC) disease (95.8%) at our hospital during 2006-2017. We calculated the prevalence of risk factors reported in Western countries among mycobacterial species. Results: Pulmonary MAC disease cases comprised 78.2% of pulmonary NTM patients in their 40s, increasing to 100% at age ≥80 years. Body mass index (BMI) was <18.5 in approximately 40% of patients, which was significantly higher than the prevalence of underweight in the Japanese population. The percentage of male heavy smokers (Brinkman index ≥600) was 58.2% of pulmonary NTM disease and was high for all mycobacterial species. In pulmonary MAC disease, systemic factors were observed in the order of malignant tumors (other than lung cancer), diabetes, rheumatoid arthritis, and tuberculosis. Local factors were observed in the order of bronchiectasis, chronic obstructive pulmonary disease, lung cancer, and bronchial asthma. Conclusion: The risk factors reported in Western countries were relatively highly prevalent among Japanese pulmonary NTM disease patients. This observation may help elucidate disease onset mechanisms.

Pathological differentiation of chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia.

AIMS: To evaluate the histological characteristics differentiating chronic hypersensitivity pneumonitis (chronic HP) with a usual interstitial pneumonia (UIP)-like pattern from idiopathic pulmonary fibrosis (IPF)/UIP. METHODS AND RESULTS: Surgical lung biopsy specimens from 22 patients with chronic HP diagnosed as having a UIP-like pattern upon histological examination and 13 patients with IPF/UIP were examined and the incidences of bronchiolitis, perilobular fibrosis, centrilobular fibrosis, bridging fibrosis, organizing pneumonia, fibroblastic foci, honeycombing, granulomas, giant cells, lymphocytic alveolitis and lymphoid follicles were compared. Bronchiolitis, centrilobular fibrosis, bridging fibrosis, organizing pneumonia, granulomas, giant cells and lymphocytic alveolitis were significantly more frequent among patients with chronic HP than among patients with IPF (all P<0.01). CONCLUSIONS: Centrilobular fibrosis, bridging fibrosis and organizing pneumonia, in addition to bronchiolitis, granulomas and giant cells, are characteristic features of chronic HP with a UIP-like pattern. These features are therefore important in differentiating chronic HP from IPF/UIP, as management strategies differ for the two disorders.

Imaging Findings of Fibrosis in Pulmonary Sarcoidosis.

Background: In pulmonary sarcoidosis, respiratory tract lesions almost always appear, and residual lung shadows require treatment in about 20% of cases. Pulmonary fibrosis is among the three leading causes of death. Treatment strategies are urgently needed to inhibit the progression of pulmonary fibrosis by combining antifibrotic drugs and immunosuppressive drugs such as corticosteroids. Establishing consensus on the process of pulmonary fibrosis progression is important for determining the most effective treatment. Our review: Among more than 2500 cases of sarcoidosis treated at our hospital, cases that led to chronic respiratory failure were analyzed for CT findings of pulmonary fibrosis. Early in sarcoidosis, granulomatous lesions appeared along the bronchovascular bundle. As pulmonary fibrosis progressed, a central consolidation developed on the central side in the direction of lymph flow, a peripheral consolidation developed on the pleural side, and a central-peripheral band developed connecting the two. Infiltrative or wedge-shaped shadows sometimes formed in the immediate subpleural area, appearing as a pleuroparenchymal fibroelastosis-like lesion. Traction bronchiectasis may form cysts at the periphery or may congregate to form a honeycomb lung-like structure. Combination of these lesions led to shrinkage of the upper lobe. Patients with multiple peripheral cysts/bullae had a unique disease course characterized by wheezing and concomitant pulmonary hypertension and pulmonary aspergillosis. Conclusion: Further understanding of the process of pulmonary fibrosis progression is needed. Summarizing imaging findings and understanding their contribution to respiratory impairment will contribute to comprehensively evaluating the stages of pulmonary fibrosis progression and establishing an optimal treatment strategy.

Paradoxical Formation of a Pleuroparenchymal Fibroelastosis-like Lesion in the Chronic Course of Pulmonary Sarcoidosis.

We herein report the long-term changes in chest computed tomography (CT) findings from early sarcoidosis lesions to pleuroparenchymal fibroelastosis (PPFE)-like lesions in a 30-year-old man with granulomas on a transbronchial lung biopsy. Multiple bilateral micronodular and nodular opacities around the bronchovascular bundle in the upper lobes detected by chest CT in 2004 disappeared, but paradoxically, peripheral consolidations continued to grow at the periphery of the original lesions. Chest CT in 2017 confirmed the progression of bilateral shrinkage of the upper lobe, spread of peripheral consolidations and wedge-shaped opacities below the first rib, and bronchiectatic air bronchograms, confirming PPFE-like lesions.

Systemic Adverse Effects Induced by the BNT162b2 Vaccine Are Associated with Higher Antibody Titers from 3 to 6 Months after Vaccination.

OBJECTIVE: We aimed to determine the relationship between vaccine-related adverse effects and antibody (Ab) titers from 3 to 6 months after the second dose of the BNT162b2 coronavirus disease 2019 (COVID-19) mRNA vaccine (Pfizer/BioNTech) in Japan. METHODS: We enrolled 378 healthcare workers (255 women and 123 men) whose Ab titers were analyzed 3 and 6 months after the second dose in our previous study and whose characteristics and adverse effects were collected previously by using a structured self-report questionnaire. RESULTS: The workers' median age was 44 years. Although injection-site symptoms occurred with almost equal frequency between the first and second doses, systemic adverse effects, such as general fatigue and fever, were significantly more frequent after the second dose than after the first dose. Multivariate analysis showed that fever was significantly correlated with female participants for the second dose (odds ratio (OR), 2.139; 95% confidence interval (95% CI), 1.185-3.859), older age for the first dose (OR, 0.962; 95% CI, 0.931-0.994) and second dose (OR, 0.957; 95% CI, 0.936-0.979), and dyslipidemia for the first dose (OR, 8.750; 95% CI, 1.814-42.20). Age-adjusted Ab titers at 3 months after vaccination were 23.7% and 23.4% higher in patients with a fever than in those without a fever after the first and second dose, respectively. In addition, age-adjusted Ab titers at 3 and 6 months after the second dose were, respectively, 21.7% and 19.3% higher in the group in which an anti-inflammatory agent was used than in the group without the use of an anti-inflammatory agent. CONCLUSION: Participants with systemic adverse effects tend to have higher Ab titers from 3 to 6 months after the second dose of the BNT162b2 vaccine. Our results may encourage vaccination, even among people with vaccine hesitancy related to relatively common systemic adverse effects.

Honeycomb-like structures in sarcoidosis pathologically showing granulomas in walls of clustered bronchioles.

In the clinical setting, it is often difficult to judge whether honeycomb-like structures represent progression of fibrosis in pulmonary sarcoidosis or a complication by interstitial pneumonitis. This report described a valuable case in which pathology of video-assisted thoracoscopic surgery specimens collected from the lungs with honeycomb-like structures that were continuous with the dilated bronchioles on chest computed tomography (CT) showed granulomas in the membranous bronchiole walls, thereby demonstrating that the honeycomb-like structures were lung lesions of sarcoidosis. Pathological features of these structures on chest CT included cystic changes attributable to incorporation of peripheral alveoli into dilated bronchioles in lobules: these findings in lung sarcoidosis were different from those corresponding to honeycomb lung in idiopathic pulmonary fibrosis/usual interstitial pneumonia. Radiological and pathological findings showed the possibility that progressive clustering of dilated bronchi and bronchioles causes cystic changes, resulting in the formation of honeycomb-like structures as fibrosis progresses in sarcoidosis with lung involvement.

Progression of Central-peripheral Band and Traction Bronchiectasis Clusters Leading to Chronic Respiratory Failure in a Patient with Fibrotic Pulmonary Sarcoidosis.

We herein report a rare case of pulmonary sarcoidosis leading to chronic respiratory failure with restrictive ventilatory impairment during a 53-year-long observation period. Nine years after the histological diagnosis of stage I sarcoidosis on chest X-ray in a woman in her 20s, she developed bilateral reticular and granular opacities on chest computed tomography and was started on prednisone for 18 years. Seven years after prednisone withdrawal, these persisting opacities around the bronchovascular bundle, including a central-peripheral band, had progressed, forming traction bronchiectasis clusters and peripheral cysts, some of which developed continuously at the distal side of these clusters, with eventual upper lobe shrinkage.

Attenuation of Antibody Titers from 3 to 6 Months after the Second Dose of the BNT162b2 Vaccine Depends on Sex, with Age and Smoking Risk Factors for Lower Antibody Titers at 6 Months.

OBJECTIVE: We aimed to determine antibody titers at six months and their percentage change from three to six months after the second dose of the BNT162b2 coronavirus disease 2019 (COVID-19) mRNA vaccine (Pfizer/BioNTech) and to explore clinical variables associated with titers in Japan. METHODS: We enrolled 365 healthcare workers (250 women, 115 men) whose three-month antibody titers were analyzed in our previous study and whose blood samples were collected 183 ± 15 days after the second dose. Participant characteristics, collected previously, were used. The relationships of these factors with antibody titers at six months and percentage changes in antibody titers from three to six months were analyzed. RESULTS: Median age was 44 years. Median antibody titer at six months was 539 U/mL. Older participants had significantly lower antibody titers (20s, 752 U/mL; 60s-70s, 365 U/mL). In age-adjusted analysis, smoking was the only factor associated with lower antibody titers. Median percentage change in antibody titers from three to six months was -29.4%. The only factor significantly associated with the percentage change in Ab titers was not age or smoking, but sex (women, -31.6%; men, -25.1%). CONCLUSION: The most important factors associated with lower antibody titers at six months were age and smoking, as at three months, probably reflecting their effect on peak antibody titers. However, the only factor significantly associated with the attenuation in Ab titers from three to six months was sex, which reduced the sex difference seen during the first three months. Antibody titers may be affected by different factors at different time points.

Age and Smoking Predict Antibody Titres at 3 Months after the Second Dose of the BNT162b2 COVID-19 Vaccine.

OBJECTIVE: We aimed to determine antibody (Ab) titres 3 months after the second dose of the BNT162b2 coronavirus disease-2019 (COVID-19) vaccine and to explore clinical variables predicting these titres in Japan. METHODS: We enrolled 378 healthcare workers (255 women, 123 men) whose blood samples were collected 91 ± 15 days after the second of two inoculations of the BNT162b2 COVID-19 mRNA vaccine (Pfizer/BioNTech) given 3 weeks apart. Medical histories and demographic characteristics were recorded using a structured self-reported questionnaire. The relationships between Ab titres and these factors were analysed. RESULTS: Median age (interquartile range (IQR)) of the participants was 44 (32-54) years. Median Ab titre (IQR) against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike antigen was 764 (423-1140) U/mL. Older participants had significantly lower Ab titres; median (IQR) Ab titres were 942 (675-1390) and 1095 (741-1613) U/mL in men and women in their 20s, respectively, but 490 (297-571) and 519 (285-761) U/mL in men and women in their 60-70s, respectively. In the age-adjusted analysis, the only risk factors for lower Ab titres were male sex and smoking. However, the sex difference may have arisen from the sex difference in smoking rate. Moreover, Ab titres were significantly lower in current smokers than in ex-smokers. CONCLUSIONS: The most important factors associated with low Ab titres were age and smoking habit. In particular, current smoking status caused lower Ab titres, and smoking cessation before vaccination may improve the individual efficacy of the BNT162b2 vaccine.

Pulmonary mycobacterium avium complex disease complicated by cancer: an 11-year survey at a single-center.

Objective: Pulmonary mycobacterium avium complex (MAC) disease is increasing significantly worldwide. Several studies have investigated the clinical features of pulmonary MAC disease in the setting of cancer. Here, we aimed to clarify the clinical characteristics of patients with cancer with recent onset of pulmonary MAC disease and the effect of cancer on the onset of this disease. Patients and Methods: Of the 323 consecutive Japanese patients newly diagnosed with pulmonary MAC disease at Jichi Medical University Hospital between and 2006-2017, we retrospectively reviewed 79 consecutive patients with cancer. Results: Seventeen patients had lung cancer (21.0%), while 62 had non-lung cancer. Of the 17 patients with lung cancer, 16 had adenocarcinoma of which 10 had stage I to III disease; 8 of the 10 patients had not received chemotherapy. Sixteen patients with lung cancer had a MAC infection in the ipsilateral lung. Notably, 9 of the 11 lung cancer patients who did not undergo surgery had a MAC infection in the affected lobe. Of the 39 patients with the most common types of non-lung cancer (14 had gastric cancer, 13 had colorectal cancer, and 12 had breast cancer), 22 had stage I to III disease, and 18 of these 22 had not received chemotherapy. Conclusion: Lung cancer may act as a local factor contributing to the onset of pulmonary MAC disease in the ipsilateral lung. However, the underlying mechanism by which a history of cancer might affect the onset of pulmonary MAC disease remains unclear. Further investigation into this mechanism is needed.

Characteristics of patients with a diagnosis of sarcoidosis: a comparison of the 2006 and 2015 versions of diagnostic criteria for sarcoidosis in Japan.

Objective: Histological verification of epithelioid cell granuloma is important in diagnosing sarcoidosis; tissue sampling is a worldwide requirement. In 2006, to reduce medical expenses and avoid invasive procedures, diagnostic criteria without histological verification were permitted by the Japanese government. In 2015, new diagnostic criteria, allowed clinical diagnoses based on only respiratory, ocular, and cardiac systems with at least a two-system involvement, increasing the need to sample tissue from clinically unevaluable organs in suspected sarcoidosis. This study aimed to compare the characteristics of patients who were diagnosed with sarcoidosis according to the 2006 and 2015 criteria. Materials and Methods: Using the 2015 version, we re-evaluated the characteristics of 264 patients with diagnosed or suspected sarcoidosis according to the 2006 criteria, at Jichi Medical University Hospital between 2004 and 2012 (clinical diagnosis, 84; histological diagnosis, 117; suspected sarcoidosis 63). Results: Thirty-nine patients were diagnosed with suspected sarcoidosis due to the absence of at least a two-system involvement; two patients had insufficient laboratory data suggestive of sarcoidosis. Six patients moved from suspected sarcoidosis to a histological diagnosis because of a greater leniency in the criteria for supportive findings. The 2015 diagnostic criteria excluded patients with organ involvement without a requirement for systemic steroids from the clinical diagnosis group. A case of schwannoma, erroneously placed in the clinical diagnosis group by the 2006 criteria, was reclassified according to the 2015 criteria. Conclusion: The 2015 version is preferable for clinically diagnosing sarcoidosis, even without histological specimens, and provides guidance for indications for systemic treatment.

Propionibacterium acnes-associated Sarcoidosis Possibly Initially Triggered by Interferon-alpha Therapy.

A 46-year-old woman with uveitis was referred to our respiratory diseases department in July 2018. Her medical history included transient bilateral hilar mediastinal lymphadenopathy (BHL) and multiple pulmonary nodules in May 2013 during pegylated interferon-alpha and ribavirin treatment for chronic hepatitis C infection. Five years post-treatment, chest X-ray revealed BHL and nodular recurrence. A biopsy of the subcutaneous buttock nodules revealed scattered non-caseating epithelioid granulomas with positive PAB immunohistochemical staining. This seem to be the first report of Propionibacterium acnes-associated sarcoidosis possibly initially triggered by interferon-alpha therapy. Understanding the mechanisms underlying interferon-triggered P. acnes-associated sarcoidosis may clarify the sarcoidosis immunopathogenesis.

The impact of demographic disparities in the presentation of sarcoidosis: A multicenter prospective study.

OBJECTIVE: To study how demographic differences impact disease manifestation of sarcoidosis using the WASOG tool in a large multicentric study. METHODS: Clinical data regarding 1445 patients with sarcoidosis from 14 clinical sites in 10 countries were prospectively reviewed from Feb 1, 2020 to Sep 30, 2020. Organ involvement was evaluated for the whole group and for subgroups differentiated by sex, race, and age. RESULTS: The median age of the patients at diagnosis was 46 years old; 60.8% of the patients were female. The most commonly involved organ was lung (96%), followed by skin (24%) and eye (22%). Black patients had more multiple organ involvement than White patients (OR = 3.227, 95% CI: 2.243-4.643) and females had more multiple organ involvement than males (OR = 1.238, 95% CI: 1.083-1.415). Black patients had more frequent involvement of neurologic, skin, eye, extra thoracic lymph node, liver and spleen than White and Asian patients. Women were more likely to have eye (OR = 1.522, 95%CI: 1.259-1.838) or skin involvement (OR = 1.369, 95%CI: 1.152-1.628). Men were more likely to have cardiac involvement (OR = 1.326, 95%CI: 1.096-1.605). A total of 262 (18.1%) patients did not receive systemic treatment for sarcoidosis. Therapy was more common in Black patients than in other races. CONCLUSION: The initial presentation and treatment of sarcoidosis was related to sex, race, and age. Black and female individuals are found to have multiple organ involvement more frequently. Age at diagnosis<45, Black patients and multiple organ involvement were independent predictors of treatment.

[Pulmonary mycobacteriosis caused by Mycobacterium peregrinum in a young, healthy man].

A 24-year-old male never-smoker with no contributory medical history was referred to our division because of chronic productive cough and fever. His chest CT showed pulmonary consolidation including nodular shadows around the bronchovascular bundle in both upper lobes and right middle lobe. Sputum smears were negative but cultures were positive for Mycobacterium peregrinum, twice. He was given a diagnosis of pulmonary mycobacteriosis caused by M. peregrinum and treated with clarithromycin, levofloxacin, and ethambutol. His symptoms and the infiltrates improved, and further sputum cultures were negative for mycobacteria. M. peregrinum is a species included in the M. fortuitum group, of rapidly-growing non-tuberculous mycobacteria. To the best of our knowledge, only 2 cases with lung infection caused by M. peregrinum have been previously reported, both of which occurred in patients in their 60s with underlying diseases, including low immunity due to immunosuppressants for polymyositis, or bronchiectasia. We describe a rare case of pulmonary mycobacteriosis due to M. peregrinum in a healthy man without any underlying diseases.

Honeycomb lung-like structures resulting from clustering of traction bronchiectasis distally in sarcoidosis.

Gene expression profiles of patients with progressive sarcoidosis, most of whom had evidence of fibrosis on imaging, have been reported to be similar to those of patients with inflammatory hypersensitivity pneumonitis, while expression profiles in progressive sarcoidosis did not resemble those of idiopathic pulmonary fibrosis. However, it is not known whether specific parenchymal features discerned on computed tomography (CT) imaging can predict development of fibrosis in pulmonary fibrosis. We herein describe a rare case of pulmonary sarcoidosis with honeycomb lung-like structures developing as a result of concentration of traction bronchiectasis distally, predominantly in both lower lung fields, which developed through shrinkage of consolidations comprising a "central-peripheral band" detected in a woman in her 60s, with non-caseating epithelioid granuloma. To our knowledge, this is the first case demonstrating the distinctive morphology and developmental process of honeycomb lung-like structures in fibrotic pulmonary sarcoidosis.

Computed Tomography Images of Fibrotic Pulmonary Sarcoidosis Leading to Chronic Respiratory Failure.

BACKGROUND: There is currently no consensus on the morphology of severe fibrotic pulmonary sarcoidosis, and we examined computed tomography (CT) findings and progression. METHODS: We analyzed findings in 10 consecutive patients (three men, seven women) with pulmonary sarcoidosis requiring oxygen therapy for chronic respiratory failure, who were extracted from >2500 sarcoidosis patients (three hospitals, 2000-2018). Patients with comorbidities causing chronic respiratory failure were excluded. RESULTS: Predominant findings were consolidations along the bronchovascular bundles comprising 'central-peripheral band', traction bronchiectasis, peripheral cysts/bullae, and upper lobe shrinkage. Traction bronchiectasis arose from opacities comprising 'central-peripheral band'. Clustering of traction bronchiectasis at the distal side formed honeycomb lung-like structures in three patients. Upper lobe shrinkage progressed in seven patients together with progression of consolidations, 'central-peripheral band', traction bronchiectasis clusters, and cysts, while patients without shrinkage included two patients with severe multiple cysts without traction bronchiectasis. Restrictive ventilatory impairment developed in most patients. Pulmonary hypertension (PH) was detected radiologically in five patients, and chronic progressive pulmonary aspergillosis (CPPA) in four patients. CONCLUSIONS: During progression, consolidations comprising 'central-peripheral band' progressed together with traction bronchiectasis clusters and peripheral cysts, resulting in upper lobe shrinkage. This may lead to respiratory failure with possible complications such as PH and CPPA.

Characteristics of 68 patients with clinically proven sarcoidosis based on the Japan Society of Sarcoidosis and Other Granulomatous Disorders 2015 criteria.

BACKGROUND: The presence of histologically evident epithelioid granuloma is required for the diagnosis of sarcoidosis worldwide. The Japan Society of Sarcoidosis and Other Granulomatous Disorders 2015 diagnostic criteria (JSSOG 2015 criteria) includes "clinically proven diagnosis" (involvement of at least 2 of 3 systems confirmed solely by clinical assessment) because of the frequency of sarcoidosis with ocular, cardiac, and respiratory involvement in Japan and the difficulty of obtaining specimens. Here, we describe in detail the clinical presentation of clinically diagnosed sarcoidosis. METHODS: We enrolled 68 consecutive patients with clinically diagnosed sarcoidosis (12 men, 56 women) based on the JSSOG 2015 criteria who were treated at Jichi Medical University between December 2018 and January 2000. We analyzed age at diagnosis, organ involvement, and laboratory findings. RESULTS: Age at diagnosis was unimodal in women. Ocular, splenic, cardiac, and skin involvement, and hypercalcemia were observed in 95.6%, 8.8%, 7.4%, 5.9%, and 35.0% of patients, respectively. High serum lysozyme and soluble interleukin-2 receptor (sIL-2R) levels, bilateral hilar lymphadenopathy on chest radiography, high-grade atrioventricular block or fatal ventricular arrhythmia, and bundle branch block were found in 18.8%, 48.3%, 95.6%, 5.0%, and 10.0% of patients, respectively. CONCLUSIONS: The age-specific distribution of clinically diagnosed sarcoidosis was similar to histologically diagnosed sarcoidosis in women, as previously reported. Rates of elevated serum lysozyme and sIL-2R levels were lower in this study than previously reported in histologically diagnosed patients in Japan.