Diagnostic yield of the Kveim test in sarcoidosis patients.

BACKGROUND AND AIM: Sarcoidosis is a granulomatous disorder of unknown etiology characterized by the existence of non-caseating granulomatous inflammation. Diagnosis can be challenging due to the presence of comprehensive clinical, laboratory, and radiologic manifestations. We have evaluated the diagnostic yield of the Kveim test and compared this test with the other conventional laboratory modalities. Our aim was to reach the highest level of diagnostic confidence acknowledging the absolute uncertainty in diagnosis with the current diagnostic enterprises. METHODS: Medical records of 300 sarcoidosis patients were reviewed. Patients were classified into two categories as the conventional laboratory and the Kveim test group to compare the diagnostic yield. RESULTS: Sensitivity of the Kveim test was 76.4% while the conventional laboratory tests provided a 64% diagnostic yield.  The conventional tests had a low diagnostic rate in the early disease stages. Kveim test revealed a high yield diagnosis for all stages of sarcoidosis. Integrated assessment of the two modalities reached a 96.8% sensitivity and a 94,6% specificity. CONCLUSIONS: Conventional laboratory modalities were useful for the assessment of disease activity and identification of organ involvement. Kveim test revealed a significant diagnostic yield for all stages of sarcoidosis. The lowest output was achieved in stage IV patients due to the waning of active granulomatous inflammation.  The highest diagnostic sensitivity was obtained by an integrated analysis of the conventional laboratory and the Kveim test results for all aspects of sarcoidosis.

Hearing loss in Takayasu's arteritis.

OBJECTIVES: Neurosensory hearing loss is well-documented in chronic autoimmune conditions such as systemic lupus erythematosus (SLE). However, the literature lacks data on the prevalence and characteristics of hearing impairment in Takayasu's arteritis (TAK). In this cross-sectional study, our principal objective was to systematically assess the auditory function of individuals diagnosed with TAK, against SLE patients and healthy controls (HC). METHODS: Age and gender matched TAK and SLE patients followed up in a tertiary centre along with healthy controls were included in a two-phase study. In the first phase, a questionnaire on ENT symptoms was administered to the patient (TAK: n=104 and SLE: n= 151) and HC (n=174) groups. In the second phase, patients (TAK: n=53 and SLE: n=33) and HC (n=45) underwent audiometric tests. RESULTS: The questionnaire survey revealed that both TAK and SLE patients reported hearing loss (27.9%, 25.8%, 7.4%, p<0.001), tinnitus (49%, 35.8%, 13.8%, p<0.001) and vertigo (46.2%, 33.8%, 16.7%, p<0.001) at significantly higher rates than HC. Audiometry results indicated that both TAK (30.2%) and SLE patients (18.2%) had increased hearing loss compared to HC (8.9%), however, only TAK patients were found to have significantly increased risk in age adjusted logistic regression analysis (OR= 3.915, 95%CI: 1.179-12.998, p=0.026). Hearing loss was mainly neurosensory in all groups. TAK patients were affected at both low (<6000 Hz) and high (>6000 Hz) frequencies, whereas SLE patients were affected only at high frequencies. Hearing loss was significantly associated only with older age. No association was observed with the anatomical location of vascular involvement or history of stroke. CONCLUSIONS: Our study reveals an increased prevalence of hearing loss in TAK. Further research is crucial to uncover the underlying causes.

How to recognize and manage challenging DRESS cases: Two case reports and a review of the literature.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is an uncommon systemic adverse drug reaction. Furthermore, it is a unique syndrome encompassing various manifestations of fever, facial edema, eosinophilia, atypical lymphocytosis, and organ dysfunction. Since there are no large prospective studies concerning DRESS syndrome, current treatment modalities for DRESS have been mainly determined based on various case reports and expert opinions. Corticosteroids are the mainstay of therapy after the cessation of the culprit drug. Although most cases recover within a couple of months, some may persist and even progress despite 1 mg/kg/day of prednisolone or its equivalent. We herein present two cases of severe DRESS syndrome. Both cases presented with organ dysfunction and remained unresponsive to initial treatment with 1 mg/kg/day of intravenous methylprednisolone. Therefore, plasmapheresis or pulse steroid therapy (250 mg/day methylprednisolone for 3 days) was used. In the follow-up period, the patients' clinical conditions improved dramatically without recurrence. We aimed to share our experience in recognizing and managing severe DRESS cases in this manuscript. Furthermore, we reviewed the literature in comparison with the present cases. In conclusion, plasmapheresis or pulse steroid therapy (250 mg/day of methylprednisolone for 3 days) can be used to treat difficult DRESS cases where organ failure is about to happen.