Pyrazinamide-Induced Urticaria and Angioedema: a Case Report / 대한내과학회지

Pyrazinamide (PZA) is an anti-tuberculosis drug and an essential component of the standard four-drug regimen for tuberculosis. Here, we report a case of immediate angioedema secondary to PZA administration intended for pulmonary tuberculosis treatment. A previously healthy 48-year-old woman was diagnosed with pulmonary tuberculosis and tuberculous lymphadenitis. Thirty minutes after taking the first dose of isoniazid, rifampicin, pyrazinamide, and ethambutol, the patient developed facial edema, generalized rash, and dizziness. An oral provocation test was performed on the four drugs, and 1,000 mg pyrazinamide showed a positive result characterized by 50 minutes of urticaria, angioedema, and hypotension. As the prevalence of tuberculosis increases, prescriptions for anti-tuberculosis drugs may increase as well. Clinicians should be aware of the possibility of immediate hypersensitivity as well as delayed hypersensitivity to anti-tuberculosis drugs.

Incidence of Active Tuberculosis within One Year after Tumor Necrosis Factor Inhibitor Treatment according to Latent Tuberculosis Infection Status in Patients with Inflammatory Bowel Disease

BACKGROUND: We investigated the incidence of active tuberculosis among patients with inflammatory bowel disease (IBD) treated with tumor necrosis factor (TNF) inhibitors, with or without latent tuberculosis infection (LTBI). METHODS: The study was performed at a Korean tertiary referral center between January 2011 and June 2017. In total, 740 patients with IBD who underwent LTBI screening tests and were followed-up for ≥ 1 year after TNF inhibitor treatment initiation were enrolled. LTBI was detected on the basis of tuberculin skin test results, interferon-gamma release assay results, chest X-ray findings, and previous tuberculosis treatment history. The patients were classified into LTBI (n = 84) or non-LTBI (n = 656) group. The risk of developing tuberculosis in each group was assessed on the basis of standardized incidence ratio (SIR) and 95% confidence interval (CI) for active tuberculosis. RESULTS: Mean patient age was 33.1 years, and patients with Crohn's disease were predominant (80.7%). Within 1 year after the initiation of TNF inhibitor treatment, 1 patient in the LTBI group (1/84; 1.2%) and 7 patients in the non-LTBI group (7/656; 1.1%) developed active tuberculosis. The overall 1-year incidence of tuberculosis among the patients was significantly higher than that among the general population (SIR, 14.0; 95% CI, 7.0–28.0), and SIR was not affected by LTBI status (LTBI group: 14.5, 95% CI, 2.0–102.6; non-LTBI group: 14.0, 95% CI, 6.7–29.4). CONCLUSION: Patients with IBD undergoing TNF inhibitor treatment showed a higher 1-year incidence of tuberculosis than the general population irrespective of LTBI status.

IgG4-Related Sclerosing Cholangitis Showing Natural Course of Progression to Decompensated Liver Cirrhosis / 대한췌담도학회지

Immunoglobulin G4-related disease (IgG4-RD) is characterized by extensive IgG4-positive plasma cells infiltration resulting in inflammation and fibrosis of the affected organs. Patient with IgG4-RD shows an excellent response to glucocorticoid therapy especially when given at early onset stages. IgG4-related sclerosing cholangitis (IgG4-SC) is considered biliary manifestation of IgG4-RD. The natural history and long-term prognosis of IgG4-SC is not well defined and most of the previous case series have reported short duration of follow-up. We present the case of development of decompensated liver cirrhosis from IgG4-related sclerosing cholangitis with a 6-year natural clinical course.

A Sporadic Case of Epstein Syndrome: A Rare Cause of Refractory Thrombocytopenia / 대한내과학회지

A 37-year-old female presented to our hospital with a history of bleeding episodes (excessive bleeding after tooth extraction, gum bleeding, easy bruising, and excessive menstruation) and severe thrombocytopenia (2,000/µL). She had no family history of bleeding tendency or thrombocytopenia. No peripheral lymphadenopathy or splenomegaly was noted. The patient's white blood cell count was normal; hemoglobin was 9.7 g/dL. A peripheral blood smear showed markedly decreased platelets, with occasional giant or large platelets. Bone marrow examination found increased megakaryocytes. The patient also complained of hearing difficulty; a hearing test indicated sensory-neural hearing impairment. Her thrombocytopenia was refractory to treatment with glucocorticosteroids, intravenous gamma-globulin, and danazol. In the 13 years following her initial presentation, the patient required anti-hypertensive treatment, a hearing-aid for progressive hearing loss, and started maintenance kidney dialysis. Her clinical history of refractory thrombocytopenia, progressive hearing impairment, and renal failure suggested myosin heavy chain 9 gene-related congenital syndrome (Epstein syndrome), which was confirmed by the presence of a heterozygous deletion mutation, c.221_223del, (p.Lys74del) in peripheral leukocyte deoxyribonucleic acid.