Sarcoidosis in Jordan: A Study of the Clinical Phenotype and Disease Outcome

Abstract

Objectives: This study aims to evaluate the clinical phenotypic features of sarcoidosis in a single-center academic hospital in Jordan. Patients and methods: A retrospective file review was performed at an academic medical center in Jordan that included all patients diagnosed with sarcoidosis between January 2000 and December 2018. A total of 150 patients with sarcoidosis (38 males, 112 females; mean age 47.8±11.7 years; range, 17 to 79 years) were evaluated. Clinical data extracted from the files included the sex of the patient, the age at time of diagnosis, diagnosis date, the season during which the diagnosis was established, and smoking history. Biopsy histopathology, spirometry, nerve conduction, echocardiography, and imaging reports including plain radiographs, ultrasonographic, magnetic resonance and computed tomography reports were reviewed. Data including laboratory values, medication usage, clinical outcomes, and morbidity/mortality were collected. Pulmonary function tests including spirometry and lung volumes along with the diffusing capacity for carbon monoxide were reviewed for the presence of restriction, obstruction or reduction in the diffusion capacity of carbon monoxide. Identification of extra-thoracic organ involvement was determined in each patient in accordance with the criteria suggested by the updated World Association of Sarcoidosis and Other Granulomatous Disorders. Results: A total of 77.3% of the patients were diagnosed by biopsy. One case of Lofgren's syndrome was identified. Of the patients, 18.0% had isolated pulmonary sarcoidosis, 75.3% had pulmonary and extra-pulmonary sarcoidosis and 6.7% had isolated extra-pulmonary sarcoidosis while 81.3% had respiratory symptoms, mostly shortness of breath and cough. Extra-thoracic organ involvement mostly involved the musculoskeletal system (33%) followed by the skin (20%). Female patients had more extra-thoracic involvement but the sex difference was only statistically significant for cutaneous involvement. Of the patients, 84% received treatment while 20% had disease remission during the first two years after diagnosis and 70% required treatment beyond two years after diagnosis. Conclusion: Various sarcoidosis clinical phenotypes are seen among Jordanian patients. Jordanian females are more affected by the disease and have more extra-thoracic involvement compared to male patients. A large number of the study patients received treatment.

Pulmonary Function in Pulmonary Sarcoidosis

“…Any PFT pattern can be seen in patients with sarcoidosis, such as a restrictive ventilatory defect (RVD), an obstructive ventilatory defect (OVD), reduced DLCO, or mixed ventilatory defects. The most prevalent pattern of PFT abnormality was an RVD due to parenchymal involvement, which occurred in about 45% of the patients [11]. An obstructive pattern can also be present and may be related to airway involvement caused by external compression of mediastinal disease, granulomatous tissue, or peri-bronchial fibrosis [12,13].…”

Darier-Roussy Subcutaneous Sarcoidosis: A Case-Based Review

“…Sarcoidosis is a granulomatous multifocal inflammatory condition of unexplained exact etiology, characterized by non-caseous epithelioid cell granulomas. It has variable clinical presentations and varying prognoses [1]. The disease is observed at a prevalence rate of 10-20 per 100,000 persons, with most cases diagnosed between the ages of 25 and 40 and a second peak in women greater than 50 years old [2].…”

Rebuttal From Drs Kupari and Lehtonen

“…The lungs are involved most often, usually as part of multi-organ disease but also as isolated pulmonary sarcoidosis. 1,2 Extrapulmonary single-organ involvement is also a possible though much rarer manifestation, with isolated cutaneous sarcoidosis topping the respective statistics. 3 In countering the significance of isolated cardiac sarcoidosis (CS), Birnie et al 4 emphasize the nonexistence of "truly isolated CS."…”