Legionella pneumonia typically presents as lobar pneumonia with multiple-lobe involvement, but Legionella lung abscess is rare. To identify the predisposing factors for Legionella abscess, we analyzed 62 of the 79 case reports on Legionella abscess found in literature; 28 (45.2%) were of hospital-acquired infection and 28 (45.2%), community-acquired infection. Seventeen patients (27.4%) died. L. pneumophila serogroup 1 was the most common, but other serogroups of L. pneumophila, L. micdadei, L. bozemanii, L. dumoffii, and L. maceachernii were also isolated from the abscess. Corticosteroids were administered for underlying diseases to 43 (69.4%) patients. Peripheral neutrophil counts were higher in patients with abscess than in those with only pulmonary infiltration. In certain cases, Legionella abscess developed during neutropenia recovery. However, lymphocyte counts were low in most cases. Clinical factors like corticosteroid treatment, which causes impaired cellular immunity and subsequent neutrophil accumulation in the lesion, might function as predisposing factors for Legionella abscess.
Autoimmune pancreatitis (AIP) is often associated with systemic extrapancreatic lesions, and the concept of IgG4-related autoimmune disease has been proposed. We report a 62-year-old woman with AIP associated with various extrapancreatic lesions during a long-term clinical course. These extrapancreatic lesions occurred after the withdrawal of steroid therapy. Sclerosing sialadenitis occurred before AIP, whereas autoimmune sensorineural hearing loss, allergic purpura and endocapillary proliferative glomerulonephritis occurred after AIP. However, no relapse was observed for 5 years after the start of maintenance therapy with prednisolone and azathioprine.
A 52-year-old woman was diagnosed with cap polyposis (CP) with characteristic clinical, endoscopic, and histological features. By avoiding straining at defecation, her symptoms improved temporarily, however recrudesced. She was diagnosed with Helicobacter pylori (H. pylori) infection, and received eradication therapy successfully. After this eradication therapy, her symptoms and colonoscopic findings recovered completely. Only two reports in the English language literature have discussed the relationship between CP and eradication therapy for H. pylori, all patients achieved complete recovery. We recommend H. pylori testing for all cases of CP and H. pylori eradication therapy if necessary.
Endoclip migration into the common bile duct following laparoscopic cholecystectomy (LC) is an extremely rare complication. Migrated endoclip into the common bile duct can cause obstruction, serve as a nidus for stone formation, and cause cholangitis. We report a case of cholangitis due to a migrated endoclip and consequent choledocholithiasis 6 years after LC, which was successfully treated by endoscopic extraction.
A 58-year-old Japanese woman presented with chronic fluctuating liver dysfunction with purpura. Raynaud's phenomenon had been diagnosed 4 years previously. At the initial examination, skin biopsy showed limited cutaneous systemic sclerosis (SSc). Laboratory investigations revealed liver dysfunction. Anti-nuclear antibodies, anti-mitochondria M2 antibody, anti-thyroglobulin antibody, and platelet-associated IgG were positive. Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) were diagnosed serologically, clinically and histologically. Immune thrombocytopenic purpura (ITP) was diagnosed by bone marrow puncture, clinical and laboratory findings, and Helicobacter pylori IgG was positive. She was treated with prednisolone 30 mg/day, ursodeoxycholic acid 600 mg/day, and a 7-day course of lansoprazole plus amoxicillin and clarithromycin. Thrombocytes increased rapidly and transaminase improved at day 7. We report a rare case of PBC-AIH overlap syndrome with concurrent ITP and SSc which suggest the presence of shared genetic susceptibility factors in multiple autoimmune conditions including PBC, AIH, ITP and SSc.
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) of the thymus is rare and little is known about its karyotype abnormality. MALT lymphoma in general shows a good prognosis, but some reports suggest that the presence of trisomy 18 predicts recurrence. Here, we report a patient with MALT lymphoma of the thymus and the left parotid gland accompanied by Sjogren's syndrome. The karyotype analysis revealed that this is the first case of thymic MALT lymphoma with trisomy 18, which we believe is worth reporting. We also review cases with thymic MALT lymphoma previously reported in the literature.
A 74-year-old man was administered bepridil for the treatment of atrial fibrillation since February 2008. However, he developed exertional dyspnea in October 2008. Computed tomography scans of his chest revealed extensive bilateral peribronchial consolidations. Examination of transbronchial lung biopsy specimens revealed moderate infiltration of lymphocytes. Since drug-induced pneumonia was suspected, we initiated steroid therapy. After 3 weeks of treatment, the symptoms were alleviated. In this case, the time taken for the development of dyspnea was 226 days, and the clinical course was gradual. We believe that a long-term periodic follow-up is essential in patients receiving bepridil.
The present case was an elderly man with a history of gastric cancer, diffuse biliary duct stenosis and liver cirrhosis. He had markedly elevated IgG, suggesting chronic infection or inflammatory changes in the biliary duct. He developed weakness in his arms and became unable to use his hands within one month and 2 weeks later, he had difficulty walking. Based on his progressive disease course, elevated serum IgG, nerve conduction study and enhanced MRI findings, we diagnosed him as suffering from immune-mediated subacute polyradiculoneuropathy with polyclonal gammopathy, which might be related to Guillain-Barré syndrome. Intravenous immunoglobulin (IVIg) was dramatically effective in this patient. In the follow-up 6 months later he was stable and could walk without a cane. Even in patients with polyclonal gammopathy in chronic inflammatory disease of another organ, IVIg may be effective and beneficial for the patients's quality of life.
Here, we present a 54-year-old man with proptosis and swelling below the left eyelid. Laboratory findings showed high levels of PR3-ANCA and histological examination of the first biopsy revealed acute inflammation. Together with the findings of MRI, a diagnosis of WG was made. However, the disease progressed rapidly and histological examination of the second biopsy revealed infiltration of neoplastic T lymphocytes with aberrant loss of CD7. A final diagnosis of peripheral T cell lymphoma, not otherwise specified (WHO) was made, and complete remission was achieved by chemotherapy. This is a very rare case of T cell lymphoma with a high titer of PR3-ANCA.