Category: Adrenal Disorders
Cushing’s disease (CD) is the most common cause of endogenous cortisol excess. The magnitude of cortisol excess is associated with severity of disease. We present a case of CD without classical phenotype despite high cortisol, posing a diagnostic challenge.
Methods : NA
Results : NA
A 60-year-old female with vascular dementia was hospitalized for symptomatic anemia. Three months before this hospitalization she was diagnosed with type 2 diabetes, deep venous thrombosis (DVT), pulmonary embolism and non-healing wound over right ankle. Physical exam was remarkable for multiple ecchymoses on extremities, periorbital edema and hypertension. No evidence of moon facies, supraclavicular fat pad, striae or obesity. Hospital course was complicated by retroperitoneal hematoma. Discontinuation of anticoagulant led to new DVT requiring IVC filter. CT abdomen showed an incidental finding of L1 and L2 compression fractures. Refractory hypokalemia was notable. This in the context of hypercoagulable state, compression fracture and variable insulin requirements raised suspicion of cortisol excess.
Testing indicated ACTH-dependent hypercortisolism [random cortisol 122.2 ug/dl, ACTH 154.2pg/ml, loss of circadian rhythm and failure of low-dose dexamethasone suppression test (DST) with resultant cortisol of 111.6 and 151.1 ug/dl]. A high-dose DST showed a decline in cortisol to 38.5 ug/dl and elevated ACTH 67.5pg/ml. CT chest/abdomen/pelvis were negative. CRH stimulation test demonstrated a rise in ACTH (84.9 to 366 and 75.7pg/ml at 10 and 25 minutes). MRI brain showed no pituitary enlargement. Inferior petrosal venous sinus sampling (IPSS) revealed lateralization to the right. Transsphenoidal hypophysectomy was scheduled. While awaiting surgery, patient developed pneumonia. Her clinical status deteriorated and family decided on comfort care.
Initially, ectopic hypercortisolism was suspected given our patient’s age and rapid onset of symptoms but pan-CT was negative. This patient had an acute, severe and rapidly progressing clinical presentation with multiple metabolic consequences in the absence of a typical clinical phenotype. Adequate cortisol suppression was observed with high-dose DST. An unremarkable pituitary MRI added to the clinical dilemma. It is noteworthy that 40% of CD patients have no visible tumor on standard MRI. CRH stimulation and bilateral IPSS confirmed the diagnosis.
Typically CD is considered an indolent disease compared to Cushing's syndrome due to ectopic ACTH secretion, clinical distinction is not always easy and rapidly progressing Cushing's disease results in significant morbidity and mortality without treatment.
Vidita Divan– Fellow, SUNY Upstate Medical Center, Camillus, New York
Maria Fariduddin– Resident, SUNY Upstate University Hospital
Prakash Nadkarni– Associate Professor of Medicine, SUNY Upstate Medical University
Ruban Dhaliwal– Assistant Professor of Medicine, SUNY Upstate Medical University, New York
Vidita Divan– Fellow , SUNY Upstate Medical University, Camillus, New York
SUNY Upstate Medical Center
Camillus, New York
I graduated from SMIMER (Surat Municipal Institute of Medical Education and Research) from Surat, India. I did my Internal Medicine residency training from Aurora Health Care, Milwaukee, WI (2014-2017). I was delighted to accept the position of a Chief resident at Aurora Health Care which helped to enhance my mentorship skills, time management and leadership skills. I was fortunate to start my fellowship in Endocrinology at SUNY Upstate Medical University in July 2018.
I strongly believe in Evidence based medicine and was involved in quality improvement project and committee during residency training. I also got opportunity to do poster presentation at American Geriatric Society Annual meeting (May, 2016) and Endocrine Society (March, 2018) as well as an oral presentation at American College of Physician (September,2015). I also wrote a book chapter on thyroid disease in the elderly which was published in "Encyclopedia for the Elderly".
As an Endocrine fellow, and ultimately a practicing internist, I plan on committing my life to the betterment of my patients, and the profession.
SUNY Upstate University Hospital
I am Maria, I did my medical school in India and have been training in internal medicine in Syracuse, NY. I am very passionate about endocrinology and looking to pursue a career as an endocrinologist.
Associate Professor of Medicine
SUNY Upstate Medical University
Education & Fellowships
• Fellowship: New England Medical Center, 1997, Endocrinology, Diabetes and Metabolism
• Fellowship: Baylor College of Medicine, 1996, Endocrinology, Diabetes and Metabolism
• Residency: New York Medical College, 1994, Internal Medicine
• Residency: B.Y.L. Nair Municipal Hospital, 1991, Internal Medicine
• MBBS: University of Bombay, India, 1988
Specialties & Certification
• Internal Medicine
• Endocrinology, Diabetes and Metabolism
• Endocrine Society
• College of Physicians and Surgeons, Mumbai, India
• American Association of Clinical Endocrinologists
• American Diabetes Association (ADA)
• Association of Physicians of India
• National Board of Medicine, New Delhi, India
Current Hospital Privileges
• Upstate University Hospital
• VA Medical Center
• Crouse Hospital
Languages Spoken (Other Than English)
Assistant Professor of Medicine
SUNY Upstate Medical University, New York
Education & Experience
Residency: North Shore-Long Island Jewish Health System
Fellowship (Endocrinology, Diabetes and Metabolism): SUNY Upstate Medical University
An Observational, Longitudinal, Prospective, Long-Term Registry of Patients with Hypophosphatasia. Protocol ALX-HPP-501.
Paradighm (Physicians Advancing Disease Knowledge in Hypoparathyroidism): A Natural History Registry for Patients with Chronic Hypoparathyroidism
Certifications & Licensure
American Board of Internal Medicine: Certified in Endocrinology, Diabetes and Metabolism
American Board of Internal Medicine: Certified in Internal Medicine - NY State Medical License