We studied a usefulness and validity of the Japanese guideline for diagnosis of ocular sarcoidosis and the diagnostic criteria for sarcoidosis. Guideline for ocular sarcoidosis required 3 or more of 6 ocular findings suggestive of sarcoidosis. Sensitivity of the criteria of the guideline was high, but specificity seemed to be low. Difficulty in diagnosis of sarcoidosis was reconfirmed by our previous study. In 56 patients with ocular sarcoidosis suspect, only 55% of the patients were diagnosed as sarcoidosis by the diagnostic criteria which required positive biopsy or 3 or more of 5 clinical data; increased serum γ-globulin, increased serum lysozyme, positive Ga scan including at least negative PPD test or increased serum angiotensin converting enzyme. Bronchoalveolar lavage study seemed sensitive to diagnose ocular sarcoidosis. Forty-six % of the suspected ocular sarcoidosis with negative clinical data were diagnosed by transbronchial lung biopsy. Ocular sarcoidosis consists of minute lesions, and difficulty in diagnosis may result from inactive or minimal involvement of other organ which can not be detected by routine clinical investigations.

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In the treatment of chronic sarcoidosis refractory to steroid, methotrexate and other immunosuppresive drugs have been reported as the steroid sparing altetrnatives. The issue is reviwed on the basis of Baughman's special lecture entitled “New treament of sarcoidosis”, performed at the 18th annual meeting of Japan society of sarcoidosis and other granulomatous disorders.

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We reviewed 23 cardiac sarcoidosis (CS) patients to clarify some factors leading to serious conditions and analyzed clinical problems on the treatment of congestive heart failure. In 4 patients presenting as congestive heart failure and dilated cardiomyopathy (DCM)-like condition, the diagnosis of CS was confirmed several years after the insertion of permanent pacemaker for advanced atrio-ventricular (AV) block. In spite of continuation of corticosteroid, relapse of active CS was noted. Moderate to severe mitral regurgitation occurred in 8 of the 23 patients (34%) during the clinical course. There were 15 patients (65%) with complete AV or bi-fascicular block. Patchy interstitial fibrosis was frequently observed in the biopsied myocardium. According to analysis of echocardiography, thallium-201 scintigraphy and left ventriculography, anteroseptal regions of the heart was more preferentially affected in CS. In conclusion, delayed diagnosis, relapse of active myocardial lesions, and the developement of mitral regurgitation in CS lead to serious conditions. Beta-blocker therapy and partial left ventriculotomy for severe heart failure may be difficult to be performed in CS patients because of the above mentioned clinicopathological characteristics and lesion distribution.

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A 64-year old man was admitted to the hospital because of progressive dyspnea. His chest X-ray showed diffuse reticulonodular shadows in the lower lung fields and HRCT revealed ground-galss opacities and fibrotic patterns similar to idiopathic pulmonary fibrosis. He had hypoxemia and his serum ACE was elevated. The specimen obtained at open lung biopsy showed non-necrotizing epithelioid cell granuloma with multinucleated giant cells in lung parenchyma, establishing the diagnosis of sarcoidosis. By steroid therapy his clinical and radiological findings had been improved. Two years after the initial admission he died of squamous cell carcinoma in the left upper lobe. The postmortem lung showed honeycombing in the subpleural area and slightly thickened alveolar wall in the inner area, but granulomatous lesion was unable to be found. We reported a case of sarcoidosis which had unusual course and radiological findings.

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Skin involvement of sarcoidosis was found at detection and during the course of the disease in sarcoidosis. In sarcoidosis patients with lupus pernio and other types of skin involvement, persistence of sarcoid lesion on chest X-ray film is found frequently. Heart, liver, bone and other extrapulmonary involvement persisted in some cases with skin involvement including lupus pernio.

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For the last two decades, the association between sarcoidosis and malignancy has been disputed. We evaluated the occurrence and type of malignancy on the basis of data among 249 patients with sarcoidosis for a total of 3292 personyears presenting at Okayama University Hospital. Ten patients had 14 sites and 10 types of malignancies. In those, three patients had double or triple cancer in the course of sarcoidosis.
Case 1 had chronic lymphocytic leukemia (B cell type, IgMλ) and lung cancer (squamous cell carcinoma, stage I). Case2 had gastric cancer and colon cancer. Case 3 had basal cell carcinoma of the skin on the nose, Bowen's disease of the foot and non-Hodgkin's lymphoma (diffuse, large cell type, clinical stage IV).
We found three cases developing double or triple cancer. These cases may suggest relationship between sarcoidosis and malignancy.

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A 70-years-old woman complaining blurred vision in December 1997 consulted to our ophthalmologist, who made the diagnosis of bilateral uveitis. Her serum level of lysozyme and ACE were elevated, PPD skin test was negative. Chest X-ray film revealed bilateral hilar lymphadenopathy which had been never seen before.
ECG disclosed complete right bundle brunch block (CRBBB) which had also been never recognized before. She had been managed for essential hypertention 5 years before the onset with uveitis. Skin biopsy from the right knee revealed epithelioid cell granuloma.
As myocardial sarcoidosis was suspected, coronary angiography and myocardial biopsy was performed. These procedures failed to show neither ischemic change of coronary arteries nor epithelioid cell granuloma. ²⁰¹Tl myocardial scintigram showed some defect of myocardial uptake. We diagnosed clinically myocardial sarcoidosis based on the diagnostic criteria.
Corticosteroid therapy including steroid pulse therapy failed to improve ECG findings. Then the prolonged use of low-dose methotrexate (MTX, 5mg a week) was added to corticosteroid in May 1998. The CRBBB disappeared after 6 month's treatment with MTX, and by that time the dose of prednisolone had been reduced to 10mg a day at that time.
The prolonged low-dose MTX therapy was useful for the management of steroid resistant myocardial sarcoidosis.

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A case of sarcoidosis complicated with rheumatoid arthritis (RA) is described. A 42 year old male patient presented with erythema on the auricular skin visited our hospital in August 1995. He had been diagnosed as having sarcoidosis at the age of 25, and he had no symptoms since then. Specimen taken from the ear demonstrated noncaseating epithelioid cell granuloma. RA was diagnosed by the criteria of American Rheumatism Association (1987). The possible immunological mechanisms concerning their concurrent appearance are discussed: if sarcoidosis showed imbalance between T helper 1 (Th1) and Th2, it could perhaps predispose the development of RA.

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A 64 years-old man admitted because of acute respiratory failure (PaO2 39.6 Torr) with diffuse pulmonary interstitial shadows. Clinical diagnosis was interstitial pneumonia (unknown cause), but further examination cannot be done due to severe hypoxemia. So he was treated with steroid. Hypoxemia and diffuse interstitial shadows dramatically improved. After discharge prednisolone was tapered off, but 5 months later, uveitis occurred and pulmonary involvement exacerbated with mediastinal lymphadenopathy and increase of serum ACE level (19.4IU/l). Mediastinoscopic lymph node biopsy revealed non-caseating epithelioid cell granuloma. He received steroid therapy which improved uveitis and interstitial pneumonia. Although prednisolone was taperd, cataracta that is probably side effect by steroid therapy was exacerbated and serum ACE level increased again (24.3IU/l). So cyclophosphamide (50mg/day) was administrated with prednisolone, serum ACE decreased (5.4IU/l) and eye and lung involvement improved. For a severe case of sarcoidosis with interstitial pneumonia and uveitis, corticosteroid and cyclophosphamide therapy was effective.

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Two overlapping cases of Sjögren's syndrome and sarcoidosis are reported.
Case 1, A73-year old woman with a 11-year history of primary Sjögren's syndrome, was admitted to our hospital for evaluation of fever and abnormal shadow on chest X-ray film. Laboratory data revealed ACE 39.4. Chest CT scans showed bilateral hilar lymphadenopathy with thickening of the pertbronchovascular interstinum. A transbronchial lung biopsy was performed revealing numerous epithelioid cell granuloma consistent with sarcoidosis.
Case 2, A41-year old man was admitted to our hospital for evaluation of fever and abnormal shadow on chest X-ray film. Laboratory data revealed positive anti SS-A antibody. Chest CT scans showed bilateral hilar and mediastinal lymphadenopathy with thickening of the pertbronchovascular interstinum. A transbronchial lung biopsy was performed revealing epithelioid cell granuloma consistent with sarcoidosis. A diagnosis of Sjögren's syndrome was confirmed by positive Schirmer's test and sialography, which showed an abnormal pooling with an apple tree-like pattern in the parotid gland.

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Heerfordt syndrome is a condition in which parotid gland enlargement, facial nerve paralysis and uveitis are accompanied by low grade fever, and it is considered a subtype of sarcoidosis. Although the complete type of Heerfordt syndrome exhibiting all four of these symptoms is considered rare, we encountered a patient who showed all four symptoms simultaneously.
The patient was a 24 year old man who consulted our hospital with the chief complaint of left facial nerve paralysis and worsening of blurred vision. Uveitis suggestive of sarcoidosis was noted in both eyes.
The patient had a low grade fever with a body temperature of 37°C, and enlargement and tenderness of the left parotid gland were noted. Chest X-ray films and computed tomography revealed enlargement of the hilar lymph nodes bilaterally and a reticular and granular shadow in the middle lung field, and the diagnosis of Heerfordt's syndrome was made. The tuberculum reaction converted to negative, and the serum angiotensin converting enzyme level was elevated. Biopsy of the anterior scalene node revealed epithelioid cell granuloma, confirming the diagnosis of sarcoidosis. In addition to topical treatment, tapered steroid therapy was provided, and the patient's course has been favorable.

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We report five cases with pulmonary sarcoidosis who showed clubbing, fine crackles, and interstitial opacities which should be differentiated from chronic interstitial pneumonia.
All five cases were of relatively high age (mean 52.4) at the onset and presented with fine crackles during the course of the disease. Clubbing was seen in four cases. Chest X-ray films revealed reticulonodular shadows in all cases and honeycoming in four. Serum ACE activity was elevated in four cases and BAL fluid lymphocytosis was found in two, accompanied by elevated CD4/CD8 ratio. Four cases showed restrictive lung impairment. Diagnosis of sarcoidosis was established by lymph node biopsy in three, and transbronchial lung biopsy in one case. The last case was diagnosed as having the disease by the presence of Schaumann bodies in the lungs and lymph nodes. Two cases have been observed for three and seven years, while the rest died seven, fourteen, and 21 years after the onset, respectively. The incidence of this type of disease described above was 0.6% based on our own panels of 868 patients with sarcoidosis.

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We present here two cases of sarcoidosis whose symptoms worsened or improved in accordance with the change of psychological stress loads. The first case, a forty-nine-year-old housewife, who had been treated with corticosteroid for one year, was introduced to our hospital for shortness of breath and diffuse pulmonary shadows. A close interview revealed that she had suffered from her son's violence in the home. After our counseling to her family, her medical symptoms and the chest X-ray findings decreased accompanied with the cessation of her son's violence. Though sometimes her condition reverted after a recurrence of the violence in the home, she is now in good condition without use of corticosteroid. The second case, a thirty-one-year-old male working as a waiter until midnight, was admitted to our hospital due to blurred vision. The chest X-ray films revealed diffuse granular shadows. We advised him to stop the midnight job and to lead a regular life. His symptoms attenuated after his submission to our counseling but soon recurred when he returned to a disorderly life style.
Although it might not be always true for other sarcoidosis patients, we would like to emphasize that psychological stress or a disorderly life style may worsen the condition of sarcoidosis patients, especially of those with alexithymic status.

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A case of sarcoidosis with calcium nephropathy is reported. A 62 year-old-female was admitted in September, 1996, when she had fever, renal dysfunction, hypercalcemia, abnormal X-ray findings and body weight loss. At admission, sarcoidosis was diagnosed because of TBLB finding of non-caseous epithelioid cell granuloma, negative tuberculin test, iritis with secondary glaucoma and elevated level of serum γ-globulin. Hypercalcemia and renal dysfunction improved after six days of admission with no particular treatment. Renal biopsy showed intact glomeruli, interstitial fibrosis. After discharge she had two episodes of hypercalcemia and renal dysfunction. Each renal biopsy revealed interstitial fibrosis, small granuloma near-by tubules and calcium deposit. Improvement of both hypercalcemia and renal dysfunction was obtained by only bed rest.

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We reported two patients with sarcoidosis in the same family. The patients were a seventy five-year old female (mother), and a fifty five-year old female (daughter). Bilateral hilar lymphadenopathy in the annual chest x-ray examination was related to the diagnosis. However they had insidious symptoms which were exertional dyspnea of the mother and the misty vision of the daughter. The mother showed mild hypoxemia with pulmonary peripheral hypoperfusion. The daughter showed the persistent facial plaque and bilateral uveitis. Their serum angiotensin converting enzyme (ACE) levels were normal, and their genotype of ACE was II. Common types of their HLA antigens were A31, B52 and DR2. Biopsy specimens of plural organs from both patients revealed epithelioid cell granuloma without necrosis. They were estimated to have chronic active sarcoidosis. Although the serum ACE levels may remain normal, we have to observe the activity of sarcoidosis carefully in the familial cases with the ACE genotype II.

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It is often difficult to diagnose cardiac sarcoidosis according to presently available guideline, because the detection rate on cardiac biopsy is low. We report here two cases in which cardiac sarcoidosis could not be diagnosed by guideline but was strongly suspected. In both cases serum angiotensin converting enzyme and lysozyme levels were elevated, and sarcoidosis was diagnosed by lung biopsy. In one case right axis deviation was seen, while in the other no abnormalities were apparent on ECG. Neither case showed any particular abnormalities on Holter ECG or echocardiography. However, both showed diffuse spotty decreased uptake on Thallium-201-BMIPP myocardial scintigraphy. Endomyocardial biopsy did not reveal epithelioid cell granuloma. On coronary angiography no significant stenosis was noted, and the marked spotty decreased uptake on myocardial scintigraphy strongly suggested cardiac lesion of sarcoidosis. Our cases suggest that there exist some cases in which cardiac sarcoidosis cannot be diagnosed by the presently available guidline. We believe that this is an important issue to be addressed in the area of diagnosis of cardiac sarcoidosis.

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