RESUMEN
Unusual presentations of sarcoidosis pose a diagnostic challenge and warrant attention. Hematologic associations: Case 1 (37-years-old male): Pancytopenia: myelofibrosis (leading to sepsis and mortality) following a two-year quiescent course of biopsy-proven-sarcoidosis. Case 2 : (38-years-old male): Presentation with thrombocytopenia (5 × 103/cmm): immune thrombocytopenic purpura (histologically associated with megakaryocytic emperipolesis). Biopsied enlarged lymph nodes demonstrated sarcoidosis. Hematologic sarcoid involvement is usually due to granulomatous bone marrow (3.9%) or splenic infiltration (6-30%); however, the presented manifestations are scarcely reported with a potential significance that is yet to be elucidated. Case 3: Neurologic presentation: 48-years-old female: presentation with bilateral sensorineural hearing loss and facial palsy. Brain magnetic resonance imaging showed leptomeningeal thickening. Biopsied enlarged lymph nodes showed sarcoidosis. Case 4: Neurologic and renal manifestations: 13-years-old male (family history of sarcoidosis): Presenting with acute headache, investigations showed elevated serum creatinine (2.1 mg/dL) and angiotensin converting enzyme, and computed tomography chest and abdominal findings characteristic of sarcoidosis. Associated benign increased intracranial and acute tubulointerstitial nephritis (with eosinophils) were diagnosed upon concordant workup. Of sarcoidosis neurologic affection (5-10%), cranial nerve(s) involvement is among the most common (25-50% of neurosarcoid affection), particularly that of the facial nerve (Case 3). Leptomeningeal enhancement is among the most common neurosarcoid radiologic findings (30-40%). Whereas benign increased intracranial tension (Case 4) is much less reported. Among sarcoidosis renal involvement (35-50%), interstitial nephritis usually presents with granulomatous renal lesions, yet its sole association with sarcoidosis is unusual (Case 4). The portrayed atypical hematologic, neurologic, and renal manifestations further emphasize the masquerading nature of sarcoidosis.
RESUMEN
INTRODUCTION/OBJECTIVES: Despite its importance, adherence to treatment has not been sufficiently studied in Behçet's disease (BD). The aim of this study was to evaluate medication adherence in BD using the Compliance Questionnaire of Rheumatology (CQR) and factors potentially affecting it. METHOD: This cross-sectional study included 67 consecutive BD patients including 57 (85%) males with a mean age of 35.1 ± 9.27 years and mean disease duration of 129 ± 91 months. The cumulative clinical manifestations, the Behçet's Disease Current Activity Form (BDCAF) score, and the Vasculitis Damage Index (VDI) were recorded. The CQR, Socioeconomic Status Questionnaire for Health Research in Egypt (SES), the Beliefs about Medication Questionnaire (BMQ), and the Short Form 36 (SF-36) quality of life assessment questionnaire were administered to the patients. Linear regression analysis was done to determine independent predictors of CQR. RESULTS: The mean BDCAF score was 3.27 ± 3.54 and the VDI was 3.36 ± 2.21. The mean CQR score was 69.2 ± 11.79. The CQR score varied significantly among different health sources (p = 0.02), with no relationship detected with other sociodemographic characteristics, nor with clinical characteristics or the SF-36. Among the investigated medications' complexities, severity of side effects showed significantly different CQR scores (p = 0.004), and a weak positive correlation between medications' numbers and the CQR was detected. Predictors for higher CQR scores included the necessity beliefs score of the BMQ (ß = 1.1, p < 0.001); whereas, predictors for lower CQR scores were the harm and concern BMQ subscales ((ß = - 1.5, p = 0.004) and (ß = - 0.72, p = 0.032), respectively). CONCLUSIONS: Beliefs about medications were the only predictor for adherence in our cohort.