Epithelioid cell granulomas in the lung are distributed along the lymphatic vessels, i.e. bronchovascular bundle, pleura, interlobular septa, and some alveolar walls. However, conglomerated granulomas occasionally involved alveolar lumina resulting from destruction of alveolar epithelial basement membrane. Lymphocyte alveolitis was observed mainly near granulomas. Granulomatous angiitis is one of the predominant histologic features of a lung with Sarcoidosis. Almost all the blood vessels were involved by granuloma and its scarring in the autopsied lungs.
In long-lasting pulmonary sarcoidosis, remodeling of the lung architecture due to fibrosis from granuloma occurred, i.e, upper lobe contraction, bronchovascular bundle fibrosis, scattered stellate and band-like fibrosis, honeycombing, cystic and cavitary lesions. Microangiopathy in sarcoidosis was defined as endothelial degeneration and multilamellation of basement membrane, and observed in the microvessels of bronchial mucosa and the alveolar walls. We examined experimentally induced granuloma formation in the lung by intravenous injection of P. acnes in mice. Electron microscopical examination revealed lymphocytes and monocytes attached to the endothelium of alveolar capillary and osmiophilic material in the phagosomes of the endothelium. These findings suggest that P. acnes in the lung may induce granulomas due to activation of lymphocytes and macrophages and may induce endothelial injury.

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Chronic airway infection is mainly caused by suppression of airway mucosal defense mechanism. Diffuse panbronchiolitis (DPB), one of the representative chronic airway infectious diseases is known to show association with HLA-B54 as well as SNPs in 80 kb in the HLA class I region on the human sixth chromosome. We further searched for the regulatory regions of airway mucin genes and demonstrated that a genetic polymorphism in the regulatory region of MUC5B gene was significantly associated with chronic airway diseases including DPB

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We retrospectively examined histological granulomatous lesions of skin biopsy specimens taken in our department in 2004. Eighty-eight (obtained from 35 males and 53 females) of the 879 biopsy specimens showed granulomatous lesions. The average age of the cases was 46.7 years old and the average period between the notice of eruptions and skin biopsies was 24.8 months. The most frequent findings were foreign body reactions against the skin components or exogenous materials (57%), followed by sarcoidosis, erythema nodosum, and tumors. Foreign body reactions and tumors were easily diagnosed, because of their specific histological features in addition to granulomatous reactions. Since erythema nodosum caused by various background diseases including Behçet's disease and sarcoidosis had no characteristic microscopic features, clinical and laboratory findings are important to make a diagnosis of the background diseases. The granulomatous reactions were seen not only in inflammatory disorders but also tumors, suggesting that detailed examinations are necessary in cases with an atypical clinical progress or features of inflammatory granulomas.

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A 30-year-old woman was examined with facial nerve palsy and blurred vision due to uveitis in January 1999. Chest X-ray showed bilateral hilar lymphadenopathy. Because she rejected any further examination, we started prednisolone in a dose of 30mg every day and all the symptoms improved. Transbronchial lung biopsy was performed four months after the initiation of corticosteroid therapy, and the patient was diagnosed as having sarcoidosis. In May 2003, she came to have dry cough and dyspnea. Chest X-rays showed progressive stenosis of the left main bronchus and atelectasis of the left lung. Although she was re-treated with the same dose of corticosteroids as before, the stenosis did not improve and led to complete atelectasis of the left lung. To rule out the possibilities of bronchial tuberculosis or lymphoma, videoassisted diagnostic thoracoscopy with mediastinal lymph node biopsy was carried out and the pathological findings were compatible with sarcoidosis. Here we report a rare case of sarcoidosis with unilateral pulmonary atelectasis.

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A 45-year-old man was admitted to hospital because of chest pain and dyspnea. He was diagnosed to have right pneumothorax. The pneumothorax did not improve with bed rest alone, so he was referred to our hospital. The pneumothorax was resolved with chest tube drainage. But a chest x-ray and CT showed bilateral diffuse micronodules and mediastinal lymphadenopathy. Serum ACE was elevated slightly. Bronchoalveolar lavage revealed increased lymphocytes percent with elevated CD4/CD8 ratio. Transbronchial biopsies demonstrated non-caseating epithelioid cell granulomas without infectious organism. So he was diagnosed to have sarcoidosis. After one month, right pneumothorax recurred. At this time, tube drainage treatment was again successful and then he received pleurodesis by OK-432. Pneumothorax due to sarcoidosis is relatively rare, especially as an initial manifestation.

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A 55 year-old female had swelling of lymph nodes in cervical, axillary, and inguinal regions detected by comprehensive medical checkups in October 2004 and was suspected of having malignant lymphoma at a clinic. In November, an FDG-PET examination showed abnormal uptake in the mediastinum, intrathoracic, spleen and parotic regions, in addition to the above-mentioned lymph nodes. Sensing the possibility of sarcoidosis, she was referred to our hospital. A biopsy of inguinal lymph nodes revealed non-caseating epitheloid cell granulomas compatible with sarcoidosis. Skin biopsy and TBLB also showed similar pathological findings.
Recent studies have disclosed the efficacy of FDG-PET examination of lymph node enlargement in differentiating cancer metastasis of malignant lymphoma from sarcoidosis. We report a case of lymph node enlargement suspected of being sarcoidosis found by an FDG-PET examination.

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Cardiac involvement of sarcoidosis is important because of the high risk of death. Although the diagnosis of myocardial sarcoidosis is often difficult to confirm, early diagnosis and early corticosteroid therapy are important factors to improve the prognosis of cardiac sarcoidosis. We describe herein a case of cardiac sarcoidosis with complete atrioventricular block which was improved by corticosteroid therapy. A 28-year-old Japanese man was referred to our hospital because of an abnormal ECG showing complete atrioventricular block which was detected by a heath check-up. His chest radiographs and computed tomographs revealed consolidation on the right upper lobe. Bronchoscopy was performed and transbronchial lung biopsy showed non-caseating epitheloid cell granulomas. Transthoracic echocardiogram showed the thinning of the basal portion of the ventricular septum and ^99mTc-tetorofosmin scintigram showed decreased uptake on ventricular septal, and inferior and free anterior wall. From these observations the diagnosis of cardiac sarcoidosis was made and prednisolone 30mg/day started. Thereafter, ECG abnormalities gradually improved and now his ECG shows normal sinus rhythms at a dose of 10mg a day of prednisolone. With the improvement of atrioventricular conduction disturbance, abnormalities of cardiac scintigraphy and echocardiogram also improved.

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A 28 year-old woman with facial nerve palsy was admitted to a hospital in Feb. 2003. Facial palsy was improved by corticosteroid therapy, but in May 2003, she was admitted to Fukuoka University Hospital because of her uveitis and the swelling of bilateral hilar lymphnodes found on a chest radiograph. She was diagnosed as having sarcoidosis of an incomplete form of Heerfordt symptom-complex, incomplete form, based on the following findings: Negative PPD skin test, raised serum γ-globulin, increased ratio of CD4/CD8 in bronchoalveolar lavage fluid, and transbronchial lung biopsy showing noncaseous epithelioid cell granuloma. She was then observed without corticosteroid in the outpatient clinic, and hilar lymph node swelling was improved. In Sept. 2004, skin eruptions appeared on bilateral cubital joints and face. Biopsy of the eruption on the right cubital joint was positive for epithelioid cell granulomas. In Nov. 2004, she noticed pain of the right foot and MIP joint. In Jan. 2005, she noticed increased fever, head neuralgia, and submandibular lymphnode swelling, and was re-hospitalized. Head MRI revealed central nervous system sarcoidosis, and she was treated with 60mg of oral predonisolone a day. Central nervous system sarcoid has been improving. Classification, diagnosis and treatment of neurosarcoidosis referring to previous literature are herein stated with this case report.

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We present a 47-year-old woman who was diagnosed as having uveitis in 1997. A year later she found a palpable nodule in her right leg. Histological findings of the nodule revealed non-caseating epithelioid cell granulomas, compatible with muscular sarcoidosis. She also had mediastinal and hilar lymphadenopathy and lymphocytosis with an increase of CD4/8 ratio in bronchoalveolar lavage fluid. Magnetic resonance image (MRI) showed a nodular bright signal on T2 weighted images, when enhanced by gadrinium injection. Ga-67 citrate scintigraphy showed intensely increased nodular activity in the right leg. Palpable nodules slowly increased in number and developed in all the limb muscles over a period of seven years. Angiotensin-converting enzyme and lysozyme levels, which had been within normal ranges at initial examination, rose to 74.6IU/l and 46.0μg/ml, respectively. We observed diffuse accumulation in both upper and lower extremities by ⁶⁷Ga-scintigraphy and numerous intramuscular nodules of high signal intensity by MRI of both legs. At that time, however, she did not complain of muscle weakness and atrophy. This case may indicate that nodular muscular sarcoidosis, even when spread extensively in the skeletal muscles, is unlikely to easily cause muscle weakness and atrophy.

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A 29-year old man was admitted to our hospital due to low-grade fever and abnormal pulmonary shadows in May 2001. Laboratory findings revealed an elevated serum ACE up to 39.7IU/l and uveitis was also noted. Chest X-ray film showed bilateral lymphadenopathy and patchy shadows scattered in the bilateral lung fields. A transbronchial lung biopsy (TBLB) demonstrated epithelioid cell granulomas, compatible with sarcoidosis. Serum creatinine gradually elevated although pulmonary lesions almost disappeared without treatment. A specimen of percutaneous renal biopsy demonstrated epithelioid cell granulomas with Langhans giant cells, tubular atrophy and fibrosis in the interstitium, corresponding with granulomatous interstitial nephritis. Renal function improved after corticosteroid therapy, but was not completely recovered. It should be kept in mind that granulomatous interstitial nephritis due to sarcoidosis could cause renal failure unless treatment is started at an early stage.

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In January 2005, a 25-year-old man was admitted because of bilateral eyelid swelling and narrowing of his visual field. He had multiple subcutaneous skin nodules together with enlargement of bilateral lacrimal, and parotid glands, and also cervical lymph nodes. Plain chest X-rays and CT demonstrated mediastinal and bilateral hilar lymphadenopathy. ⁶⁷Ga scintigram demonstrated accumulation in the bilateral lacrimal glands, parotid glands, bilateral hilar, and mediastinal lymph nodes, and subcutaneous nodules. Brain MRI showed nodular enhancement in pituitary stalk, but he had no neurological symptoms and aberrance of hormonal secretion. He was diagnosed as having Sarcoidosis with histological evidence of epithelioid cell granuloma in the biopsied specimens of lacrimal gland and skin. After topical corticosteroid therapy to lacrimal gland started, his symptoms remarkably improved.

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A 69-year old woman was given a diagnosis of mixed connective tissue disease, autoimmune hepatitis. Six years later, a chest CT showed multiple nodular shadows and peribronchovascular interstitial thickening in the lung without mediastinal and hilar lymph node swelling. Pulmonary sarcoidosis was suspected. Because TBLB could not confirm the diagnosis of sarcoidosis, the patient underwent video-assisted thoracoscopic surgery (VATS). The findings of the lung and intrapulmonary lymphnode on VATS showed epithelioid cell granulomas. The patient was diagnosed as having sarcoidosis according to the diagnostic criteria. In addition, the histological findings of biopsied specimens revealed fibrotic-NSIP in the lower lobe, whereas epithelioid cell granulomas were not detected. It was concluded that this case had mixed connective tissue disease-associated interstitial pneumonia. We had found a sarcoidosis case associated with mixed connective tissue disease. It has been previously proposed that connective tissue disease and sarcoidosis are related in common immunological features. Although the coexistence of Sjögren syndrome and sarcoidosis has been reported, sarcoidosis associated with mixed connective tissue disease has very rarely been reported.

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