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An 81-year-old male had been diagnosed with Waldenström macroglobulinemia (WM) eight years previously and had thus been administered appropriate treatment. Left chylothorax later developed at 3 years and 8 months after the initial diagnosis. He was hospitalized with severe anemia, general fatigue, and appetite loss one year prior to this presentation and died due to a severe fungal infection. Autopsy revealed the presence of 1,300 ml chylothorax and infiltration of lymphoplasmacytic lymphoma (LPL) cells throughout his entire body. LPL cells were found to have invaded the excitation conducting system in the heart. In an evaluation of a resected lung tissue specimen of pneumothorax, subpleural infiltrated lymphoid cells were observed to show immunohistochemical positivity for IgM and bcl-2. Although these lymphoid cells were initially considered to be non-neoplastic lymphocytes, they were later determined to be LPL cells, which thus induced dilatation and proliferation of the lymph vessels. Chylothorax complications in patients with WM are rare events and only six such cases have so far been reported. The present case is considered to be an instructive one in which autopsy suggested the invasion of LPL cells to be involved in the development of arrhythmia, pneumothorax, and chylothorax before death.

Background: Previous studies have shown that there is an association between low BALF (bronchoalveolar lavage fluid) CD4/CD8 ratio and lower remission rates in sarcoidosis patients., Aim: To investigate the patient characteristics and clinical features of Japanese sarcoidosis patients with low BALF CD4/CD8 ratios., Methods: 322 Japanese sarcoidosis patients were retrospectively analyzed, and 3 groups were selected according to BALF CD4/CD8 ratios as follows: patients with the BALF CD4/CD8 ratio in the lowest 5 percentile (Group 1: 0.43-1.41), median 5 percentile (Group II: 4.68-5.47), and top 5 percentile (Group III: 12.6-60.1). Each group consisted of 16 patients (5% of 322 patients). The patient characteristics, clinical features, and the short-term prognosis for at least 2 years (average 116 months) were compared among the groups. Multivariate analysis was performed for 322 patients to investigate the determinants of BALF CD4/CD8 ratios., Results: The number of BALF CD8+ cells were greater in Group I than in the other two groups. In Group I, there were higher incidences of younger age, male gender, and lower number of extrathoracic lesions compared with Group III. Multivariate analysis showed that younger age and male gender were independently associated with low BALF CD4/CD8 ratios. The frequency of treatment with corticosteroid and progression to pulmonary fibrosis tended to be higher in Group I., Conclusions: Low BALF CD4/CD8 ratios were due to increased number of BALF CD8+ cells. Younger age and male gender were independently associated with low BALF CD4/CD8 ratios in sarcoidosis patients.

Background: Sarcoidosis is a systemic disease characterized by T-cell activation and subsequent granuloma formation at the site of involvement. Genetic susceptibility is a key factor in the pathogenesis of this disease, and genes involved in T-cell regulation are potential candidates. Cytotoxic T-lymphocyte antigen-4 (CTLA-4) is a negative regulator of T-cell activation expressed on activated T-cells. The G allele of the CTLA-4 exon 1 polymorphism has previously been described to be associated with disease susceptibility in several autoimmune diseases. We investigated the relationship of CTLA-4 to disease susceptibility and cell profiles in bronchoalveolar lavage (BAL) in Japanese sarcoidosis patients., Methods: Japanese sarcoidosis patients (n = 135) and controls (n = 97) were typed for an A/G bi-allelic polymorphism in exon 1 of CTLA-4 using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP), and the distribution of genotypes was compared between both groups. Sixty-seven patients underwent BAL, and cell profiles in BAL fluid were compared between patients with G/G genotype and those with A/A genotype., Results: No significant differences in the distribution of genotype and allele frequencies were found between sarcoidosis (GG: 46%, AG: 39%, AA: 15%) and controls (GG: 42%, AG: 49%, AA: 8%). Patients with G/G genotype had significantly increased lymphocyte ratios, lymphocyte counts, and CD4(+) cell counts in BAL fluid compared with patients with A/A genotype (p < 0.05)., Conclusions: CTLA-4 exonl polymorphism might affect BAL fluid lymphocyte profiles in Japanese sarcoidosis patients.

Though IgA nephropathy (IgAN) and sarcoidosis have been reported simultaneously in patients occasionally, it remains controversial whether there is any association between the two. In this report we describe our experience with one definite and two subclinical cases of IgAN diagnosed during the clinical course of sarcoidosis. Our findings suggest a possible association between these disorders, and a higher incidence of subclinical IgAN among sarcoidosis patients than among the general population. We also discuss the common immunological background shared by these two diseases.

Background and Aim of the Work: Decrease in renal function was investigated in relation to altered metabolism of calcium in patients with sarcoidosis., Methods: Fifty consecutive patients with pulmonary sarcoidosis (23 men and 27 women, 47 +/- 15 years as mean +/- SD) were studied on a daily diet consisting of 500 mg of calcium and 1000 mg of phosphorus. Renal functions were evaluated with Z scores of creatinine clearance (Ccr)., Results: Decreased renal function (Ccr Z score < -1) was detected in 13 out of 50 sarcoidosis patients (26.0%). In seven patients (14.0%) Ccr Z scores were less than -2; relative risk (RR) and 95% confidence interval (95% CI) were 13.9 and 2.97 to 64.7, respectively. Ccr Z score correlated most significantly with serum concentration of ionized calcium (sCa2+) (r = -0.574, 95% CI, -0.735 to -0.352, p < 0.0001). A stepwise regression analysis adjusting for observation time, serum concentration of calcium and 1,25-Dihydroxyvitamin D, urinary excretion of calcium, and urinary calcium crystals showed that sCa2+, together with angiotensin-converting enzyme activity in serum, was the most contributory variable to decrease in renal function in sarcoidosis patients (R2 = 0.391, p < 0.0001). A cutoff value of sCa2, 1.30 mmol/L obtained with a discriminant analysis showed 69.2% of sensitivity and 89.2% of specificity for decreased renal function., Conclusions: We reported increased prevalence of decreased renal function among sarcoidosis patients. sCa2+ was a significant index that reflected the alteration of renal function.