Showing posts with label pasireotide. Show all posts
Showing posts with label pasireotide. Show all posts

Saturday, July 13, 2013

Cushing's Disease - Rare Disease Quick Facts

cushings-diagnosis


Cushing's disease is a rare condition due to excess cortisol levels that result from a pituitary tumor secreting adrenocorticotropic hormone (ACTH), which stimulates cortisol secretion.  Cushing’s disease should not be confused with Cushing’s syndrome which is increased cortisol levels but that increase can be due to any number of factors. However, Cushing’s disease is the most common form of Cushing’s syndrome.

Symptoms
The symptoms related to Cushing's disease and Cushing's syndrome are the same, since both are related to an excess of cortisol. Also, symptoms vary extensively among patients and that, with the inherent fluctuation in hormone levels make it difficult to diagnosis both conditions.

Changes in physical characteristics of the body
  • Fullness and rounding of the face
  • Added fat on back of neck (so-called "buffalo hump")
  • Easy bruising
  • Purplish stretch marks on the abdomen (abdominal striae)
  • Excessive weight gain, especially in abdominal region
  • Red cheeks
  • Excess hair growth on the face, neck, chest, abdomen and thighs
Changes in physiology/psychology
  • Generalized weakness and fatigue
  • Menstrual disorder
  • Decreased fertility and/or sex drive
  • High blood pressure that is often difficult to treat
  • Diabetes mellitus
  • Mood and behavior disorders
Diagnosis
The early stages of Cushing's disease may be difficult to recognize. However, if it is suspected, diagnosis is generally a 2 stage process. First to determine if cortisol levels are high, and if so, why they are high.

Tests to confirm high cortisol levels:
  • 24-hour urine cortisol
  • Dexamethasone suppression test (low dose)
Tests to determine cause:
  • Blood ACTH level
  • Brain MRI
  • Corticotropin-releasing hormone test
  • Dexamethasone suppression test (high dose)
  • Petrosal sinus sampling
Treatment
Surgery
  • Most patients with Cushing’s disease undergo surgery to remove the pituitary adenoma offers.
  • If the tumor is isolated to the pituitary, cure rates of 80-85% are common.
  • If the tumor has spread to nearby organs, cure rates of 50-55% are common.
Medicine (approved orphan drugs)
  • Approved for patients with Cushing’s disease for whom pituitary is not an option or surgery has been ineffective.
  • Signifor is a somatostatin receptor agonist that leads to inhibition of ACTY secretion (and subsequently decreased cortisol levels).
  • Approved for patients with Cushing’s syndrome who have type 2 diabetes or glucose intolerance and have failed surgery (or not candidates for surgery).
  • Korlym is a glucocorticoid receptor antagonist which in turn blocks the effects of the high levels of cortisol in the body. Korlym is used to treat high glucose levels due to elevated cortisol.
Medicines used but not indicated for Cushing’s disease include
Radiation
  • Radiation therapy may be used in some patients and can be very effective in controlling the growth of these tumors.
Prognosis
In most cases, treatment can cure Cushing’s disease. If not treated properly, the chronic hypercortisolism can lead to excess morbidity and mortality due to increased cardiovascular and other risk factors.

For more information

Images courtesy of the open access journal Orhanet Journal for Rare Diseases.  Castinetti et al. Orphanet J Rare Dis. 2012 7:41   doi:10.1186/1750-1172-7-41

Wednesday, July 3, 2013

Cushing’s Syndrome is Hazardous to Your Health

morbidity

People with Cushing’s syndrome, even when treated, have higher morbidity and mortality rates that comparable controls. That is the conclusion of a new study published in the June issue of the Journal of Clinical Endocrinology Metabolism. The study by Olaf Dekkers et al, examined data records from the Danish National Registry of Patients and the Danish Civil Registration System of 343 patients with benign Cushing’s syndrome of adrenal or pituitary origin (i.e., Cushing’s disease) and a matched population comparison cohort (n=34,300).  Due to the lengthy delay of many patients being diagnosed with Cushing’s syndrome, morbidity was investigated in the 3 years before diagnosis while  morbidity and mortality were assessed during complete follow-up after diagnosis and treatment.

The study found that mortality was twice as high in Cushing’s syndrome patients (HR 2.3, 95% CI 1.8-2.9) compared with controls over a mean follow-up period of 12.1 years. Furthermore, patients with Cushing’s syndrome were at increased risk for:
  • venous thromboembolism (HR 2.6, 95% CI 1.5-4.7)
  • myocardial infarction (HR 3.7, 95% CI 2.4-5.5)
  • stroke (HR 2.0, 95% CI 1.3-3.2)
  • peptic ulcers (HR 2.0, 95% CI 1.1-3.6)
  • fractures (HR 1.4, 95% CI 1.0-1.9)
  • infections (HR 4.9, 95% CI 3.7-6.4).
The study also found that this increased multimorbidity risk was present before diagnosis indicating that it was due to cortisol overproduction rather than treatment.

Many of the Cushing’s syndrome patients underwent surgery to remove the benign tumor. For this group, the investigators performed a sensitivity analysis of the  long-term mortality and cardiovascular risk in this  subgroup (n=186)  considered to be cured after operation (adrenal surgery and patients with pituitary surgery in combination with a diagnosis of hypopituitarism in the first 6 months after operation).  The risk estimates for mortality (HR 2.31, 95% CI 1.62-3.28), venous thromboembolism (HR 2.03, 95% CI 0.75-5.48), stroke (HR 1.91, 95% CI 0.90-4.05), and acute myocardial infarction (HR 4.38, 95% CI 2.31-8.28) were also increased in this subgroup one year after the operation.

The standard treatment for endogenous Cushing’s syndrome is surgery. This past year, Signifor (pasireotide) was approved for treatment of adults patients with Cushing’s disease for whom pituitary surgery is not an option or has not been curative.  Cushing’s disease, which accounts for the majority of Cushing’s syndrome patients, is defined as the presence of an ACTH producing tumor on the pituitary grand. In the study by Dekker’s et al, the percentage of patients with Cushing’s disease is not known. We look forward to reexamination of this dataset in a few years following the introduction of more treatment options for Cushing’s disease as well as an analysis that explores the differences in mortality/morbidity rates in the different subsets of patients that make of Cushing’s syndrome (Cushing’s disease, ectopic Cushing’s syndrome, Exogenous Cyshing’s syndrome).

References
Dekkers OM, Horvath-Pujo, Jorgensen JOL, et al, Multisystem morbidity and mortality in Cushing’s syndrome: a cohort study. J Clin Endocrinol Metab 2013 98(6): 2277–2284. doi: 10.1210/jc.2012-3582

Sunday, June 23, 2013

Pasireotide for the treatment of Cushing's disease

Posted online on June 17, 2013.
 (doi:10.1517/21678707.2013.807731)

Annamaria Colao Chiara Simeoli Monica De Leo Alessia Cozzolino Rosario Pivonello
Department of Clinical Medicine and Surgery, Section of Endocrinology, Federico II University, Via Sergio Pansini 5, 
80131 Naples
, Italy +39 0817462132; +39 0815465443; colao@unina.it
Author for correspondence


Introduction: Pasireotide, a novel multireceptor targeted somatostatin analog is the first drug approved for treatment of adult patients with Cushing's disease (CD) for whom pituitary surgery is not an option or has not been curative.

Areas covered: The review describes published data on efficacy and safety of pasireotide in CD patients. In particular, the review focuses on a Phase III study (CSOM230B2305) evaluating the outcomes of treatment with pasireotide at the doses of 600 and 900 µg twice daily for 12 months in 162 CD patients. This clinical trial reported a decrease in urinary free cortisol levels in the majority of patients, with a substantial reduction in nearly half and a normalization in > 25% of patients included in the study, accompanied by an improvement in clinical picture as well as a significant reduction in pituitary tumor size. Hyperglycemia appears as the most important side effect, requiring a careful monitoring and a prompt administration of glucose-lowering medications.

Expert opinion: Pasireotide seems to have a promising role as medical option for CD patients who experienced a failure or not candidate for neurosurgery; its employment will probably induce in the near future significant changes in the therapeutic approach to CD.

Read More:  http://informahealthcare.com/doi/abs/10.1517/21678707.2013.807731

Tuesday, June 18, 2013

Research and Markets: Pituitary ACTH Hypersecretion (Cushing's Disease) - Pipeline Review Report, H1 2013 Edition

'Pituitary ACTH Hypersecretion (Cushing's Disease) - Pipeline Review, H2 2013', provides an overview of the indication's therapeutic pipeline. This report provides information on the therapeutic development for Pituitary ACTH Hypersecretion (Cushing's Disease), complete with latest updates, and special features on late-stage and discontinued projects. It also reviews key players involved in the therapeutic development for Pituitary ACTH Hypersecretion (Cushing's Disease).
Scope
- A snapshot of the global therapeutic scenario for Pituitary ACTH Hypersecretion (Cushing's Disease).
- A review of the Pituitary ACTH Hypersecretion (Cushing's Disease) products under development by companies and universities/research institutes based on information derived from company and industry-specific sources.
- Coverage of products based on various stages of development ranging from discovery till registration stages.
- A feature on pipeline projects on the basis of monotherapy and combined therapeutics.
- Coverage of the Pituitary ACTH Hypersecretion (Cushing's Disease) pipeline on the basis of route of administration and molecule type.
- Key discontinued pipeline projects.
- Latest news and deals relating to the products.
Companies Involved in Pituitary ACTH Hypersecretion (Cushing's Disease) Therapeutics Development
  • Isis Pharmaceuticals, Inc.
  • Ipsen S.A.
  • Novartis AG
  • HRA Pharma, SA
  • Cortendo Invest AB
Drug Profiles: Product Description, Mechanism of Action and R&D Progress
  • LCI-699
  • mifepristone
  • ISIS-GCCRRx
  • Inhibitors of ACTH receptor
  • ketoconazole
  • Next Generation Cortisol Inhibitor
  • pasireotide Long Acting Release






Read more here: http://www.heraldonline.com/2013/06/17/4952623/research-and-markets-pituitary.html#storylink=cpy

Monday, March 18, 2013

The Latest Cushing's News Items ~ 3/18/2013


News:

Thursday, March 14, 2013

Signifor® (Pasireotide) is now Commercially Available


November 7, 2012 The US Food and Drug Administration's (FDA) Endocrinologic and Metabolic Drugs Advisory Committee (EMDAC) voted unanimously in support of the use of Signifor® (pasireotide) for the treatment of patients with Cushing's disease who require medical therapeutic intervention. The recommendation was based on data from clinical trials of pasireotide, including PASPORT-CUSHINGS (PASireotide clinical trial PORTfolio - CUSHING'S disease), the largest randomized Phase III study to evaluate a medical therapy in patients with Cushing's disease.

Patient Assistance for SIGNIFOR support for patients includes:
-          Therapy-specific support programs for out-of-pocket costs
-          Alternative assistance searches and referrals to Federal and State assistance programs
-          Referrals to Independent Charitable Foundations for assistance with co-pay costs
-          Patient assistance for low-income and uninsured patients

For more information, or to speak to a Patient Assistance NOW Endocrinology representative, please call 1-877-503-3377 (select option #3 for SIGNIFOR) Monday to Friday 8 am - 8 pm ET.

Monday, December 17, 2012

US FDA approves Novartis' Signifor for first medication to treat Cushing's disease

Basel

Monday, December 17, 2012, 16:00 Hrs  [IST]

 

The US Food and Drug Administration (FDA) has approved Novartis' Signifor (pasireotide) injection for the treatment of adult patients with Cushing's disease for whom pituitary surgery is not an option or has not been curative. 

 

Signifor is the first medicine to be approved in the US that addresses the underlying mechanism of Cushing's disease, a serious, debilitating endocrine disorder caused by the presence of a non-cancerous pituitary tumour which ultimately leads to excess cortisol in the body.

 

This approval follows a unanimous recommendation from the FDA Endocrinologic and Metabolic Drugs Advisory Committee (EMDAC) in support of the use of Signifor.

 

"The FDA approval of Signifor for Cushing's disease brings a novel pituitary-directed therapy to patients with limited treatment options," said Hervé Hoppenot, president, Novartis Oncology. "Today's milestone reinforces Novartis' commitment to addressing unmet needs and advancing treatments for rare pituitary-related disorders."

 

Cushing's disease most commonly affects adults as young as 20 to 50 years and affects women three times more often than men. It may present with weight gain, central obesity, a round, red full face, severe fatigue and weakness, striae (purple stretch marks), high blood pressure, depression and anxiety. Cushing's disease can cause severe illness and death with mortality up to four times higher than in the healthy population.

 

The approval is based on data from PASPORT-CUSHINGS (PASireotide clinical trial PORTfolio - CUSHING'S disease), the largest randomized Phase III study to evaluate a medical therapy in patients with Cushing's disease. Results from the PASPORT-CUSHINGS study found that a decrease in mean urinary-free cortisol (UFC), the key measure of biochemical control of the disease, was sustained during the treatment period in most patients with a subset of patients reaching normal levels. The study also showed that certain clinical manifestations of Cushing's disease tended to improve.

 

"Patients with Cushing's disease may suffer from debilitating manifestations, and there are many serious health complications associated with the disease," said Mary Andrews, CEO and Co-Founder of the US non-profit, The MAGIC Foundation. "The FDA approval of Signifor offers the option of a medical therapy that may help certain patients with Cushing's disease."

 

In April 2012, the European Commission approved Signiforfor the treatment of adult patients with Cushing's disease for whom surgery is not an option or for whom surgery has failed. Other worldwide regulatory filings for pasireotide for this use are also underway.

Signifor (pasireotide) is approved in the US for the treatment of adult patients with Cushing's disease for whom pituitary surgery is not an option or has not been curative, and in the European Union for the treatment of adult patients with Cushing's disease for whom surgery is not an option or for whom surgery has failed.

 

For the treatment of Cushing's disease, Signifor has been studied as a twice-daily subcutaneous (sc) injection and is currently being evaluated as a long-acting release (LAR), once-monthly intramuscular (IM) injection as part of a global Phase III program in Cushing's disease and acromegaly. Signifor is a multireceptor targeting somatostatin analog that binds with high affinity to four of the five somatostatin receptor subtypes (sst 1, 2, 3 and 5).

 

From http://pharmabiz.com/NewsDetails.aspx?aid=72752&sid=2

Tuesday, December 4, 2012

Mifepristone: is there a place in the treatment of Cushing's disease?

Carmichael JD, Fleseriu M.

Source

Cedars-Sinai Medical Center, 8700 Beverly Blvd, Los Angeles, CA, 90048, USA, john.carmichael@cshs.org.

Abstract

The purpose was to review the use of mifepristone in the treatment of Cushing's syndrome (CS) in the context of other recently published studies. We review the use of mifepristone, as published in the recent Study of the Efficacy and Safety of Mifepristone in the Treatment of Endogenous Cushing's Syndrome (SEISMIC). We also review the multiple case reports and case series of mifepristone use in CS. A review of other medications used in the treatment of Cushing's disease (CD), including pasireotide and cabergoline also provides context for the discussion of the role of mifepristone in the treatment of CD. The results show that the treatment of CD has been primarily surgical with medical therapy reserved for adjuvant therapy when primary treatment fails or other therapies require time for optimal efficacy. Two recent large prospective studies, using pasireotide and mifepristone provide new clinical insights to the medical treatment of CD in particular. Mifepristone has been used to treat excessive cortisol production by blocking the action of cortisol at the level of the glucocorticoid receptor. Until recently, the majority of clinical experience with mifepristone on the treatment of excess cortisol was derived from case reports and small case series. Based on the SEISMIC study, mifepristone was FDA approved for hyperglycemia associated with CS. In conclusion the role of mifepristone in the treatment of CD remains one of adjuvant therapy. Its place among other choices for medical therapy has yet to be firmly established and an evidenced-based approach toward the use of novel medications in the treatment of CD has not been made. Selection of medication depends on drug approval and availability in individual countries and requires cautious assessment of potential adverse effects, consideration of patient comorbidities, and efficacy.

 

PMID: 23192246 [PubMed - as supplied by publisher]

Tuesday, October 2, 2012

Novartis launches pasireotide for Cushing’s disease

Cushing’s disease patients for whom surgery is not a viable option have a new treatment alternative, in the form of the orphan medicine pasireotide.

Launched by Novartis as Signifor, the drug is a somatostatin analogue that blocks the release of excessive adrenocorticotropic hormone, thus reducing cortisol levels.

Pasireotide is available as a solution for injection that should be self-administered by the patient twice a day. Patients should be informed how to inject the drug under the skin and that using the same injection site for two consecutive injections is not recommended.

After two months of treatment, patients’ blood cortisol levels are measured to determine whether treatment should continue, and at what dose (see Panel).

Patients who experience adverse reactions may need to temporarily lower their twice-daily dose, with decrements of 0.3mg suggested in the summary of product characteristics.

Pasireotide is indicated for adults only and is contraindicated for use by patients with severe liver impairment. It should be used with caution by patients who are taking medicines that prolong the QT interval, and clinical monitoring of heart rate is recommended for patients receiving pasireotide concomitantly with bradycardic drugs.

Dose adjustments of ciclosporin, insulin and antidiabetic medicines may be required if these are taken concomitantly with pasireotide.

The EMA granted marketing authorisation for pasireotide in April 2012 on the basis that, although the proportion of patients who responded to treatment in clinical trials was small (around 15 per cent), a partial response may be of benefit to patients whose condition cannot be managed with surgery.

Product information

Class: Somatostatin analogue

Dose: 0.6mg by subcutaneous injection twice daily. After two months of treatment, patients whose urinary free cortisol levels have reduced can continue treatment for as long as they experience a benefit. The dose can be increased to 0.9mg as long as the 0.6mg dose is well tolerated. Patients who have not responded after two months of treatment should be considered for discontinuation. The recommended twice-daily dose for patients with moderate liver impairment is 0.3mg initially, up to a maximum of 0.6mg.

NHS list price: 60 x 0.3mg/ml, £2,800; 60 x 0.6mg/ml, £3,240; 60 x 0.9mg/ml, £3,240

Legal category: POM 

From PJOnline

 

Thursday, May 31, 2012

Pasireotide in Cushing's Disease

N Engl J Med 2012; 366:2134-2135 May 31, 2012

 

To the Editor:

In their study, Colao et al. (March 8 issue)1 examined the clinical efficacy and safety of two different doses of subcutaneous pasireotide in patients with newly diagnosed, persistent, or recurrent Cushing's disease. Since alternative therapies (including bilateral adrenalectomy) are available for patients with persistent or recurrent Cushing's disease, it would be important to consider all options before embarking on what might turn out to be many years of medication.

Giovanni Targher, M.D.
University of Verona, Verona, Italy 

No potential conflict of interest relevant to this letter was reported.

1 References

To the Editor:

The phase 3 trial by Colao et al. showed the efficacy of 12 months of treatment with subcutaneous pasireotide (600 or 900 μg twice daily) in patients with Cushing's disease. We now report results after 7 years of treatment with pasireotide administered as part of a phase 2 study.1 In July 2004, a 43-year-old woman with Cushing's disease, whose 24-hour urinary free cortisol level was 9.2 times the upper limit of normal, began 15 days of treatment with subcutaneous pasireotide (600 μg twice daily) that resulted in normalization of these levels (Figure 1AFIGURE 1Effects of Pasireotide Treatment on 24-Hour Urinary Free Cortisol Levels and on Adrenocorticotropin Hormone Levels during Desmopressin-Stimulation Testing.). When treatment was halted for 35 days, urinary free cortisol levels increased. In September 2004, she resumed treatment with pasireotide (600 μg twice daily), which led to clinical improvement (i.e., a weight loss of 13 kg, regular menstrual cycles, and reduced hirsutism). Hyperglycemia ensued (glycated hemoglobin, 5.7 to 7.7%), and weakness necessitated a temporary reduction in the dose to 450 μg twice daily (November 2004 to October 2005). Since November 2005, when the patient resumed taking the 600-μg dose twice daily, urinary free cortisol levels have remained in the normal range at most monthly assessments. Basal and desmopressin-stimulated adrenocorticotropin levels also decreased as a result of treatment with pasireotide (Figure 1B). To date, she has not had any serious adverse events. This case illustrates the long-term efficacy of pasireotide without the development of resistance to the drug's effects.

Rossella Libé, M.D.
INSERM Unité 1016, Paris, France

Lionel Groussin, M.D., Ph.D.
Université Paris Descartes, Paris, France

Jérôme Bertherat, M.D., Ph.D.
Hôpital Cochin, Paris, France 

Drs. Libé and Bertherat report being investigators for studies of pasireotide in Cushing's disease funded by Novartis. No other potential conflict of interest relevant to this letter was reported.

1 References

Author/Editor Response

We concur with Targher's implication that the advantages and disadvantages of all management options should be considered for each patient before a specific treatment is advised.

Libé and colleagues present a very interesting case of a patient with Cushing's disease in the extension of a phase 2 study of pasireotide. This patient was treated with pasireotide for a much longer duration than the 1 year reported in the phase 3 study.

Annamaria Colao, M.D., Ph.D.
University of Naples Federico II, Naples, Italy

Mario Maldonado, M.D.
Novartis Pharma, Basel, Switzerland

Since publication of their article, the authors report no further potential conflict of interest.

 

From http://www.nejm.org/doi/full/10.1056/NEJMc1204078

Wednesday, April 25, 2012

Novartis drug Signifor approved in the EU as the first medication to treat patients with Cushing's disease

* Reuters is not responsible for the content in this press release.

Wed Apr 25, 2012

Novartis International AG / Novartis drug SigniforR approved in the EU as the first medication to treat patients with Cushing's disease . Processed and transmitted by Thomson Reuters ONE. The issuer is solely responsible for the content of this announcement.

Signifor is first targeted approach for Cushing's disease,

  • a debilitating endocrine disorder caused by an underlying pituitary tumor that triggers excess cortisol[1],[2],[3]
  • Majority of patients in the Phase III clinical trial experienced a rapid and sustained decrease in mean cortisol levels with a subset of patients achieving normalization
  • With reduced cortisol levels, key clinical manifestations of the disease improved, including reductions in blood pressure, cholesterol, weight and body mass index[1]

Basel, April 25, 2012 - Novartis announced today that the European Commission has approved SigniforR (pasireotide) for the treatment of adult patients with Cushing's disease for whom surgery is not an option or for whom surgery has failed[1]. Signifor is the first medicine to be approved in the European Union (EU) targeting Cushing's disease.

The approval is based on data from the largest randomized Phase III study to evaluate a medical therapy in patients with Cushing's disease, a disorder caused by excess cortisol in the body due to the presence of a non-cancerous pituitary tumor[1],[2],[3]. In the study, mean urinary-free cortisol (UFC) levels were normalized in 26.3% and 14.6% of the 162 patients randomized to receive Signifor 900µg and 600µg subcutaneous (sc) injection twice daily, respectively, at month six. The primary endpoint, the proportion of patients who achieved normalization of UFC after six months without dose up-titration relative to randomized dose, was met in patients treated with 900µg twice daily[4].

In addition, the study showed the majority of the patients remaining on the study at month six (91 out of 103 patients; 88%) had any reduction in their mean UFC[5]. The median reduction in mean UFC was 47.9% in both dose groups. Reductions in UFC were rapid and sustained through the end of the study, with the majority of patients experiencing a decrease within the first two months[4].

Overall reductions in the clinical manifestations of Cushing's disease, including blood pressure, total cholesterol, weight and body mass index, were observed at months six and twelve in patients with both full and partial mean UFC control, with the greatest reductions observed in patients with normalized UFC levels[1],[4].

"As the first therapeutic option to specifically target Cushing's disease, Signifor has the potential to redefine treatment of this debilitating disease," said Hervé Hoppenot, President, Novartis Oncology. "By focusing research efforts on our understanding of this rare disease where there is significant unmet need, we have been able to successfully bring a novel treatment option to patients in the European Union."

Cushing's disease most commonly affects adults as young as 20 to 50 years and affects women three times more often than men. It may present with weight gain, central obesity, a round, red and full face, severe fatigue and weakness, striae (purple stretch marks), high blood pressure, depression and anxiety[2],[3],[6],[7].

"Patients with Cushing's disease often struggle with a variety of debilitating health issues associated with the overproduction of cortisol and previously were faced with a treatment approach limited to surgery," said Ellen van Veldhuizen, board member of the Dutch Adrenal Society. "The approval of pasireotide as a new treatment option that may help patients with Cushing's disease is welcome news."

The decision follows the positive opinion the Committee for Medicinal Products for Human Use (CHMP) adopted for Signifor in January 2012 for the treatment of Cushing's disease and applies to all 27 EU member states, plus Iceland and Norway. Signifor has orphan drug designation for Cushing's disease, a condition which affects no more than five in 10,000 people in the EU, the threshold for orphan designation[8],[9]. Additional regulatory submissions for pasireotide for the treatment of Cushing's disease are under way worldwide.

About Cushing's disease
Cushing's syndrome is an endocrine disorder caused by excessive cortisol, a vital hormone that regulates metabolism, maintains cardiovascular function and helps the body respond to stress. Cushing's disease is a form of Cushing's syndrome, in which excess cortisol production is triggered by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma. It is a rare but serious disease that affects approximately one to two patients per million per year. The first line and most common treatment approach for Cushing's disease is surgical removal of the tumor[2],[3],[10].

About PASPORT-CUSHINGS
PASPORT-CUSHINGS (PASireotide clinical trial PORTfolio - CUSHING'S disease) is a prospective, randomized, double-blind, Phase III study conducted at 68 sites in 18 countries. The study evaluated the efficacy and safety of Signifor in 162 adult patients with persistent or recurrent Cushing's disease and UFC levels greater than 1.5 times the upper limit of normal (ULN), as well as in patients with newly diagnosed Cushing's disease who were not candidates for surgery[4].

Patients with primarily moderate to severe hypercortisolism were randomized to receive Signifor sc injection in doses of 900µg (n=80) or 600µg (n=82) twice daily. The primary endpoint was the proportion of patients who achieved normalization of UFC after six months without dose up-titration relative to randomized dose, which was met in patients treated with 900µg twice daily[4].

About Signifor (pasireotide)
Signifor (pasireotide) is approved in the European Union (EU) for the treatment of adult patients with Cushing's disease for whom surgery is not an option or for whom surgery has failed. For the treatment of Cushing's disease, Signifor has been studied as a twice-daily subcutaneous (sc) injection and is currently being evaluated as a long-acting release (LAR), once-monthly intramuscular (IM) injection as part of a global Phase III program. Signifor is a multireceptor targeting somatostatin analog (SSA) that binds with high affinity to four of the five somatostatin receptor subtypes (sst 1, 2, 3 and 5)[1],[3],[11].

Information about Novartis clinical trials for pasireotide can be obtained by healthcare professionals at www.pasporttrials.com.

Important Safety Information about Signifor 
Signifor is contraindicated in patients with hypersensitivity to the active substances in Signifor or to any of the excipients and in patients with severe liver impairment.

Alterations in blood glucose levels have been frequently reported in healthy volunteers and patients treated with Signifor. Glycemic status should be assessed prior to starting treatment with Signifor. Patients need to be monitored for hyperglycemia; if hyperglycemia develops, the initiation or adjustment of antidiabetic treatment is recommended. Dose reduction or treatment discontinuation should be considered if uncontrolled hyperglycemia persists. After treatment discontinuation, glycemic monitoring (e.g. FPG or HbA1c) should be done according to clinical practice.

Monitoring of liver function is recommended prior to starting treatment with Signifor and after one, two, four, eight and twelve weeks during treatment and thereafter as clinically indicated. Therapy should be discontinued if the patient develops jaundice, other clinical signs of significant liver dysfunctions, sustained AST (aminotransferases) or ALT (alanine aminotransferase) increase five times the upper limit of normal (ULN) or greater, or if ALT or AST increase three times ULN with concurrent bilirubin elevation greater than two times ULN.

Patients with cardiac disease and/or risk factors for bradycardia need to be closely monitored. Caution is to be exercised in patients who have or may develop QT prolongation. Hypokalemia or hypomagnesemia must be corrected prior to initiating therapy and monitored thereafter. Electrocardiography should be performed prior to the start of Signifor therapy and as clinically indicated thereafter.

Treatment with Signifor leads to rapid suppression of adrenocorticotropic hormone (ACTH) secretion in Cushing's disease patients. Patients need to be monitored and instructed how to monitor for signs and symptoms of hypocortisolism. Temporary exogenous steroid (glucocorticoid) replacement therapy and/or dose reduction or interruption of Signifor therapy may be necessary.

Monitoring of gallbladder and pituitary hormones is recommended prior to initiating treatment and periodically thereafter.

Signifor should not be used during pregnancy unless clearly necessary. Breast feeding should be discontinued during treatment with Signifor.

Signifor may affect the way other medicines work, and other medicines can affect how Signifor works. Caution is to be exercised with the concomitant use of drugs with low therapeutic index mainly metabolized by CYP3A4, bromocriptine, cyclosporine, anti-arrhythmic medicines or drugs that may lead to QT prolongation.

The most frequently reported adverse events (AE) (>10%) by investigators for Signifor were diarrhea, nausea, hyperglycemia, cholelithiasis, abdominal pain, diabetes mellitus, injection site reactions, fatigue and increased glycosylated hemoglobin (HbA1c), with most events being Grade 1-2. The tolerability profile of Signifor was similar to that of other somatostatin analogs with the exception of the greater degree of hyperglycemia[1].

Disclaimer
The foregoing release contains forward-looking statements that can be identified by terminology such as "potential," "under way," or similar expressions, or by express or implied discussions regarding potential future marketing approvals for Signifor or regarding potential future revenues from Signifor. You should not place undue reliance on these statements. Such forward-looking statements reflect the current views of management regarding future events, and involve known and unknown risks, uncertainties and other factors that may cause actual results with Signifor to be materially different from any future results, performance or achievements expressed or implied by such statements. There can be no guarantee that Signifor, or its LAR version, will be approved for sale, or for any additional indications, in any market, or at any particular time. Nor can there be any guarantee that Signifor will achieve any particular levels of revenue in the future. In particular, management's expectations regarding Signifor could be affected by, among other things, unexpected regulatory actions or delays or government regulation generally; unexpected clinical trial results, including unexpected new clinical data and unexpected additional analysis of existing clinical data; government, industry and general public pricing pressures; the company's ability to obtain or maintain patent or other proprietary intellectual property protection; competition in general; unexpected manufacturing issues; the impact that the foregoing factors could have on the values attributed to the Novartis Group's assets and liabilities as recorded in the Group's consolidated balance sheet, and other risks and factors referred to in Novartis AG's current Form 20-F on file with the US Securities and Exchange Commission. Should one or more of these risks or uncertainties materialize, or should underlying assumptions prove incorrect, actual results may vary materially from those anticipated, believed, estimated or expected. Novartis is providing the information in this press release as of this date and does not undertake any obligation to update any forward-looking statements contained in this press release as a result of new information, future events or otherwise.

About Novartis
Novartis provides innovative healthcare solutions that address the evolving needs of patients and societies. Headquartered in Basel, Switzerland, Novartis offers a diversified portfolio to best meet these needs: innovative medicines, eye care, cost-saving generic pharmaceuticals, preventive vaccines and diagnostic tools, over-the-counter and animal health products. Novartis is the only global company with leading positions in these areas. In 2011, the Group's continuing operations achieved net sales of USD 58.6 billion, while approximately USD 9.6 billion (USD 9.2 billion excluding impairment and amortization charges) was invested in R&D throughout the Group. Novartis Group companies employ approximately 124,000 full-time-equivalent associates and operate in more than 140 countries around the world. For more information, please visit http://www.novartis.com.

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References
[1] SigniforR (pasireotide) Summary of Product Characteristics. Basel, Switzerland: Novartis; April 2012.
[2] National Endocrine and Metabolic Diseases Information Service. US National Institutes of Health. Cushing's Syndrome. Available at:http://endocrine.niddk.nih.gov/pubs/cushings/Cushings_Syndrome_FS.pdf. Accessed March 2012. 
[3] Pedroncelli, A. Medical Treatment of Cushing's Disease: Somatostatin Analogues and Pasireotide. Neuroendocrinology. 2010;92(suppl1):120-124.
[4] Colao, A. A 12-Month Phase III Study of Pasireotide in Cushing's Disease. New Engl J Med. 2012; 366:32-42.
[5] Tritos N., Biller, B. Advances in Medical Therapies for Cushing's Syndrome. Discovery Medicine. 2012:13(69):171-179.
[6] Newell-Price, J., et al. The Diagnosis and Differential Diagnosis of Cushing's Syndrome and Pseudo-Cushing's States. Endocrine Reviews.1998;19(5):647-672.
[7] Bertanga, X., et al. Cushing's Disease. Best Practice & Research Clinical Endocrinology & Metabolism. 2009;23:607-623.
[8] European Commission. The Centralised Procedure. Available at:http://ec.europa.eu/health/authorisation-procedures-centralised_en.htm. Accessed March 2012.
[9] European Medicines Agency. Committee for Orphan Medicinal Products. Public Summary of Positive Opinion for Orphan Designation of Pasireotide for the treatment of Cushing's Disease. Available at:http://www.ema.europa.eu/docs/en_GB/document_library/
Orphan_designation/2009/10/WC500006176.pdf
. Accessed March 2012.
[10] Lindholm, J., et al. Incidence and Late Prognosis of Cushing's Syndrome: A Population-Based Study. J Clin Endocrinol Metab. 2001;86(1):117-23.
[11] US National Institutes of Health. Efficacy and Safety of Pasireotide Administered Monthly in Patients With Cushing's Disease. Available at: http://clinicaltrials.gov/ct2/show/NCT01374906. Accessed March 2012.

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Media release (PDF)

From http://www.reuters.com/article/2012/04/25/idUS52612+25-Apr-2012+HUG20120425

Saturday, March 24, 2012

New drug shows promise in patients with rare illness

Two in Montreal with Cushing's disease were among 16 in international trial

By AARON DERFEL, The Gazette

Quebecers suffering from Cushing's disease - a rare hormonal disorder - have some reason to be hopeful after a clinical trial conducted partly in Montreal has shown promising results of a new drug.

About 400 to 500 Quebecers have Cushing's disease or syndrome, a disorder that produces tumours on the pituitary gland, leading to a spike in cortisol levels.

The excess cortisol can cause a wide range of problems, including obesity, hypertension and diabetes as well as sleep and mood disorders.

In some people, deposits of fat accumulate on the back of the neck and shoulders, an effect known as a "buffalo hump."

Until now, surgery and radiotherapy have been the only options for many patients. However, a drug developed by Novartis Pharmaceuticals has been found to act on the tumours, cutting cortisol levels an average of 50 per cent.

In some patients - two of them from Quebec - cortisol levels returned to normal.

The drug, pasireotide, involves twice-daily injections. The treatment has yet to be approved by Health Canada.

"This study is very promising, especially for patients for whom complete surgical removal of tumours - the standard treatment for this disease - was not possible," said Dr. André Lacroix, an endocrinologist at the Centre hospitalier de l'université de Montréal.

Lacroix and his colleagues tested the drug on four patients. In addition to the two who "experienced a complete regression of all symptoms" of Cushing's disease, the two others reported drops in their cortisol levels and an improvement in their health, Lacroix noted.

The findings of the international study of 16 patients were published in the latest edition of the New England Journal of Medicine.

Pasireotide acts by binding to certain receptors on the tumours. Lacroix's team has also carried out a separate study of another drug that targets a different receptor.

He suggested that using the two drugs together might prove even more beneficial, but this must be borne out by further research.

The ideal patients for the drug therapy would be those whose tumours are too small for surgery, Lacroix said.

Each year in Quebec, there are about 15 new cases of Cushing's disease, and doctors at the CHUM treat about 150 patients.

aderfel@montrealgazette.com

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drug+shows+promise+patients+with+rare+illness/6340316/story.html