Simultaneous development of sarcoidosis and cutaneous vasculitis in a patient with refractory Crohn's disease during infliximab therapy.

BACKGROUND: Paradoxical inflammations during anti-TNF-α therapy are defined as adverse effects such as psoriasiform skin lesions, uveitis and sarcoidosis-like granulomas induced by immune reactions, not by infectious agents. Here, we report a very rare case of the simultaneous development of sarcoidosis and cutaneous vasculitis in a patient with refractory Crohn's disease during infliximab therapy and both of which resolved spontaneously without the cessation of infliximab. CASE PRESENTATION: In September 2000, 23-year old Japanese male was diagnosed with Crohn's disease. Prednisolone in combination with mesalazine was introduced at first and succeeded for almost one year. In June 2002, since his gastrointestinal symptoms relapsed and were refractory, infliximab (IFX) therapy 5 mg/kg was introduced. In February 2011, because he had repeated arthralgia almost every intravenous IFX administration, IFX was increased to 10 mg/kg under the diagnosis of a secondary failure of IFX. In December 2012, he complained of slight dry cough and an itchy eruption on both lower limbs, and he was referred to our hospital due to the appearance of bilateral hilar lymphadenopathy on chest X-ray examination. Chest computed tomogram revealed bilateral hilar lymphadenopathy and fine reticulonodular shadows on the bilateral upper lungs. Serum calcium, angiotensin-converting enzyme and soluble interleukin 2 receptor levels were not elevated, but the titer of antinuclear antibody was considerably elevated. Mycobacterium infection was carefully excluded. Trans-bronchial lung biopsy showed non-caseating epithelioid cell granulomas compatible with sarcoidosis. The skin biopsy of the right limb was diagnosed as leukocytoclastic vasculitis. The patient was diagnosed as having a series of paradoxical inflammations during anti-TNF-α therapy. Since his paradoxical inflammations were not severe and opportunistic infections were excluded, IFX was cautiously continued for refractory Crohn's disease. Nine months later, not only his intrathoracic lesions but also his cutaneous lesions had spontaneously resolved. CONCLUSION: Physicians caring for patients with anti-TNF-α therapy should know that, based on a careful exclusion of infectious agents and thoughtful assessment of the patient's possible risks and benefits, paradoxical inflammations can be resolved without the cessation of anti-TNF-α therapy.

[Clinical analysis of home oxygen therapy in patients with pneumoconiosis at Iwamizawa Rosai Hospital].

Although home oxygen therapy (HOT) has become one of the standard therapies in the management of chronic respiratory failure, especially for patient with hypoxia, there are very few studies about HOT in patients with pneumoconiosis. Therefore, we clinically investigated the characteristics of 97 patients with pneumoconiosis and 30 patients with COPD on home oxygen therapy (HOT) followed at Iwamizawa Rosai Hospital during the period from 1990 to 2003. The mean survival time in patients with pneumoconiosis (42 months) by the Kaplan-Meier method was shorter than in patents with COPD (75 months). Among the patients with pneumoconiosis divided by chest radiography classification of pneumoconiosis, there was no significant difference in mean survival time. The survival time in pneumoconiosis patients with dyspnea grade IV was shorter than in patients with dyspnea grade III.

Antithetical effect of tumor necrosis factor-alphagene polymorphism on coal workers' pneumoconiosis (CWP).

BACKGROUND: Inter-individual variation in the severity of pneumoconiosis has been described, even with the same environmental exposure. We hypothesized that TNF-alpha promoter polymorphisms associate with lung responses to environmental exposure in coal worker's pneumoconiosis (CWP) patients. METHODS: We examined polymorphisms at -238, -308, and -376 in 124 patients with CWP who had similar dust exposure history and in 122 non-exposed controls. CWP patients were divided into two groups: (1) nodular CWP (n = 84); (2) progressive massive fibrosis (PMF) (n = 44). RESULTS: The -308 A allele frequency was higher in patients with CWP compared to controls (6.35% and 2.05%, P < 0.01). It was also higher in patients with nodular CWP compared to PMF (P < 0.05). Logistic regression analysis revealed that patients with the -308 A allele were 3.8 times (P = 0.036) and those with smoking habit were 2.3 times (P < 0.002) more likely to have nodular CWP than PMF. CONCLUSION: TNF-alpha-308 A allele might interact with smoking to enhance susceptibility to nodular CWP.

Diagnostic usefulness of bronchoalveolar lavage in Hermansky-Pudlak syndrome: a case with double lung cancers.

A 65-year-old man was admitted to our hospital because of dyspnea on exertion. He had oculocutaneous albinism innately and his parents were consanguineous. His chest roentgenogram on admission showed reticulo-nodular infiltrates and cystic changes throughout both lung fields, and 7 cm mass in the left middle field. Cytology of bronchoalveolar lavage fluid (BALF) revealed macrophages containing ceroid. The diagnosis of HPS was made clinically and the tumor was diagnosed as poorly differentiated adenocarcinoma of the lung. He died of respiratory failure. By autopsy, additional well-differentiated adenocarcinoma was detected. Cytology of BALF was useful to confirm ceroid accumulation in the lung.