Journal des maladies pulmonaires et du traitement

Rationale: Management of sarcoidosis patients with the potential for multi-organ involvement can be complex and require the expertise of multiple specialties.
Objectives: Our goal was to estimate the current number of clinics dedicated to the management of patients with sarcoidosis, and to evaluate what clinic structures and definitions currently exist. Methods: A link to an online survey was emailed to Division Directors of Pulmonary and Critical Care Programs at academic medical centers in the United States. The survey email included 126 of the 142 U.S. academic pulmonary and critical care programs. The National Inpatient Sample database (2010) was used to identify yearly hospitalizations for sarcoidosis patients by state, as a surrogate indicator of prevalence of sarcoidosis across the United States.
Measurements and main results: A total of 40 (31.8%) Pulmonary Division Directors responded to the survey. Our survey results suggest a minority of academic medical centers have dedicated sarcoidosis clinics (40%), and that the existence of a dedicated sarcoidosis clinic is associated with the number of sarcoidosis patients seen annually. Only three centers (7.5%) reported having a multidisciplinary sarcoidosis clinic in which providers from different specialties see sarcoidosis patients concurrently. Multidisciplinary sarcoidosis clinics appeared to be located where hospitalization rates for sarcoidosis are higher.
Conclusions: A minority of academic medical centers has a dedicated sarcoidosis clinic, and the minority of dedicated sarcoidosis clinics used a concurrent multidisciplinary model. Additional research comparing patient populations, clinic organization and processes, as well as patient outcomes is needed to determine the optimal clinic structure for sarcoidosis. We assert that the sarcoidosis community should create a standard multidisciplinary model for evaluation, diagnosis, and treatment which could lead to more reliable conclusions and progress in treatment of sarcoidosis.

Idiopathic Lymphoid Interstitial Pneumonia (LIP) is considered to be a rare interstitial lung disease. The initial American Thoracic Society/European Respiratory Society (ATS/ERS) International multidisciplinary consensus paper on Idiopathic Interstitial Pneumonia of 2002, LIP was classified as one of the seven major idiopathic interstitial lung diseases. Since the first description of LIP in 1969, so far only 14 biopsy proven cases of idiopathic LIP have been described in the literature. The clinical course is reported to be highly variable in this rare form of interstitial pneumonia.

Introduction: Dantrolene is a muscle relaxant used to treat malignant hyperthermia as well as muscle spasms in spinal cord injury patients. It is known to cause a decrease in skeletal muscle strength in humans, and inhibit diaphragm muscle strength in hamsters. There is at least one other case report of dantrolene leading to respiratory failure in a spinal cord injury patient. Here was present a case of a patient developing poor mucus clearance, lobar collapse and respiratoy failure after being started on dantrolene, and trend vital capacity and negative inspiratory force while titrating off dantrolene.
Case: 47 y/o male with history of motor vehicle accident resulting in tetraplegia 19 months prior to admission, presented with pneumonia for the first time since his accident. He also experienced recurrently episodes of bilateral lung collapse and respiratory failure and was found to have significant respiratory muscle weakness. He had been started on dantrolene approximately six weeks prior to admission. After stopping dantrolene his clinical status and his vital capacity and negative inspiratory force dramatically improved. He was then able to clear his secretions, and had no further episodes of lung collapse.
Discussion: Our case is only the second in literature of dantrolene causing respiratory failure in a spinal cord patient; and is the first to trend daily vital capacity and NIF while the patient is being tapered off the dantrolene. Our case documented a dramatic improvement NIV and VC after stopping dantrolene. Our case demonstrates the potential and avoidable harm of using dantrolene in group of patients for which is commonly prescribed.