Drug Rashes

Pharmacy | Johns Hopkins US Family Health Plan

Drug Rashes | Johns Hopkins Medicine

Johns Hopkins USFHP utilizes the TRICARE® pharmacy formulary. The formulary lists all of the prescription drugs that are covered under the TRICARE benefit. It is a tiered, open formulary, and includes generic drugs (Tier 1), preferred brand drugs (Tier 2), and non-preferred brand drugs (Tier 3). Each of these tiers represents a cost share that USFHP members are responsible for paying.

Use the TRICARE formulary search tool to find coverage and cost share details for a specific drug.

You can also find information about medications, including how to take them, possible side effects, and drug interactions. Registration is required for first-time users. Log in and see “Health Resources”.

Formulary changes

The formulary is updated on a regular basis to include tier changes and utilization (UM) requirements. Review the latest formulary changes.

Prior authorization

Some medications require prior authorization from our plan before they can be dispensed by your pharmacist. This helps us ensure that your prescriptions are medically necessary.

For a list of drugs that require prior authorization, refer to the TRICARE Prior Authorization page.

To initiate a prior authorization, your doctor must complete and fax the prior authorization form to the Johns Hopkins HealthCare Pharmacy department at 410-424-4037.

Please note: If another Health Plan or Tricare has previously approved a medication, USFHP will not have access to that information. If you or your provider have a copy of the previous approval letter, please fax it along with the Prior Authorization Form to USFHP.

Step therapy

Step therapy is a process where we look for ways to provide our members the most cost-effective medication that is safe and clinically effective for their condition.

The preferred prescribed medication is often a generic version that offers the best overall value in terms of safety, effectiveness, and cost.

Non-preferred drugs are only prescribed if the preferred medication is ineffective or poorly tolerated.

Drugs subject to step therapy will be approved for first-time users only after they have tried one of the preferred agents as covered in the TRICARE formulary. When medically necessary, your doctor can request an exception to the step therapy requirement by submitting a prior authorization request.

Quantity limits

Quantity limits are established for certain drugs to ensure the medication is being used correctly. If your medical condition warrants a larger quantity of your medication than the listed quantity limit, your doctor should submit a prior authorization request.

Co-Pay reduction for non-formulary drugs

If your doctor can establish that you are not able to be treated with generic or preferred formulary brand medications, you can get non-formulary drugs at a network pharmacy, or through home delivery.

Your doctor can request a co-pay reduction on your behalf by completing and submitting a non-formulary co-pay reduction request form.

If the requested drug also requires prior authorization, your doctor should submit a prior authorization request as well.

Source: https://www.hopkinsusfhp.org/members/my-benefits/pharmacy/

Drug Rashes

Drug Rashes | Johns Hopkins Medicine

Linkedin Pinterest Rashes and Skin Inflammation

Drug rashes are the body's reaction to a certain medicine. The type of rash that happens depends on the medicine causing it and your response.

Medicines have been linked to every type of rash, ranging from mild to life-threatening. The timing of the rash can also vary. It may appear right away or a few weeks after you first take the medicine.

Rashes caused by medicines can be put into one of 3 groups:

  • Rashes caused by an allergic reaction to the medicine
  • Rashes as an unwanted side effect of a certain medicine
  • Rashes from extreme sensitivity to sunlight caused by the medicine

What are the different types of rashes caused by medicines?

Type of rashSymptomsCause
Acne Pimples and red areas that appear most often on the face, shoulders, and chest Anabolic steroids, corticosteroids, bromides, iodides, and phenytoin
Exfoliative dermatitis Red, scaly skin that may thicken and peel and involve the entire body Antibiotics that contain sulfa, barbiturates, isoniazid, penicillins, and phenytoin
Fixed drug eruption A dark red or purple rash that reacts at the same site Antibiotics and phenolphthalein (found in certain laxatives)
Hives Raised red bumps Aspirin, certain medicine dyes, penicillins, and many other medicines
Morbilliform or maculopapular rash A flat, red rash that may include pimples similar to the measles Antibiotics, antihypertensives, and contrast dye are among more common medicines, but any medicine can cause this rash
Purpuric eruptions Purple areas on the skin, often on the legs Some anticoagulants and diuretics
Stevens-Johnson syndrome Blisters or a hive- rash on the lining of the mouth, vagina, or penis that can spread all over the body Antibiotics that contain sulfa, barbiturates, penicillins, and certain medicines used for seizures and diabetes

How are drug rashes diagnosed?

Diagnosing a rash caused by a reaction to medicine is complicated. First, a complete review of all prescription and over-the-counter medicines should be done. Even a small amount of a medicine can cause a major reaction in the skin.

In addition, the reaction can occur even after you have taken a medicine for a long time. Your healthcare provider will usually advise you to stop taking any medicine that is not needed to sustain your life, to see if the reaction eases. Your provider may give you a substitute medicine, if possible.

In some cases, a skin biopsy may be done to help with the diagnosis. 

Treatment for drug rashes

The condition usually clears up if you stop taking the medicine that is causing the reaction. Other treatment may include:

  • Corticosteroids
  • Antihistamines

Allergic reactions can be serious and even fatal. If a rash develops, it is important to contact your healthcare provider right away. 

Source: https://www.hopkinsmedicine.org/health/conditions-and-diseases/drug-rashes

Curant Health And Johns Hopkins Medicine Launch First-Ever Study Measuring Impact of Medication Therapy Management Outcomes For IBD Patients

Drug Rashes | Johns Hopkins Medicine

September 30, 2014 – Atlanta, GA – Curant Health, provider of enhanced medication therapy management and specialty pharmacy services, is launching a study with the Meyerhoff Inflammatory Bowel Disease Center of the Johns Hopkins University School of Medicine to “implement and evaluate the effectiveness of an Inflammatory Bowel Disease (IBD) Medication Therapy Management (MTM) patient fulfillment model compared to standard care in a large university hospital setting.”

Project “A.L.I.V.E” (Adherence and Long-term IBD Value-added Effectiveness) was announced this morning during MedCity ENGAGE, a gathering of healthcare professionals in Bethesda, Md.

According to Principal Investigator and Assistant Professor of Medicine at Johns Hopkins University Dr.

Sharon Dudley-Brown, Crohn’s disease (CD) and ulcerative colitis (UC) are chronic, debilitating conditions that can have important economic and clinical implications.

In 2004, the annual cost of IBD in the United States was estimated at $1.84 billion. IBD is associated with high morbidity, loss of work productivity and impaired quality of life.

According to the study abstract, the absence of an integrated MTM platform in IBD care limits the ability to track and fully understand patient outcomes including Adverse Drug Events (ADE’s), hospital readmissions and long-term adherence rates. Systematic review of the literature validates there is no reported long term adherence data for CD or UC.  wise, no data has been published evaluating the impact of MTM on IBD outcomes.

“Our enhanced medication therapy management services are proven to reduce readmissions rates and improve adherence to medication regimens for chronically ill patients, the most difficult and costly to treat,” says Patrick Dunham, President and CEO of Curant Health. “Continuing to validate our work alongside Dr. Dudley-Brown and her colleagues, and most importantly improving the lives of people suffering from chronic conditions IBD, is central to our mission.”

During each MTM session with the patient, pharmacists will complete a medication reconciliation, identify any medication related issues, monitor therapy and provide patient education on the importance of adherence with treatment and provider follow-up, instructions on how to take/administer medications and side effects of medications.

The team will evaluate results at 30 days, 60 days, six months, nine months and 12 months from the initiation of the IBD MTM patient fulfillment model.

About Curant Health

Curant Health is a recognized leader in the field of enhanced medication therapy management, improving patients’ outcomes and lives while lowering the cost to treat chronic, high-expense conditions, including HIV, HCV, diabetes, hypertension and COPD. Twice recognized as one of America’s Fastest Growing Companies by Inc. Magazine, Curant was one of 10 finalists in Microsoft’s Excellence in Innovation competition for its development and deployment of its EMR system, MedPlan.

Health systems currently employing Curant’s services include University of New Mexico Truman Health Services, the University of Alabama at Birmingham, Emory Midtown ID Clinics, University of Louisville Research Foundation, Virginia Commonwealth University and others.

About Johns Hopkins Medicine

Johns Hopkins Medicine (JHM), headquartered in Baltimore, Maryland, is a $6.7 billion integrated global health enterprise and one of the leading health care systems in the United States. JHM unites physicians and scientists of the Johns Hopkins University School of Medicine with the organizations, health professionals and facilities of The Johns Hopkins Hospital and Health System.

Source: https://curanthealth.com/curant-health-and-johns-hopkins-medicine-launch-first-ever-study-measuring-impact-of-medication-therapy-management-outcomes-for-ibd-patients/

Johns Hopkins Medicine, Author at Drug Discovery and Development

Drug Rashes | Johns Hopkins Medicine

The first study of the immunotherapy drug pembrolizumab as the initial treatment for patients with a rare but aggressive form of skin cancer known as Merkel cell carcinoma reports better responses and longer survival than expected with conventional chemotherapy. The study, co-led by Suzanne Topalian, M.D., associate director of the Bloomberg~Kimmel Institute for Cancer Immunotherapy…

Johns Hopkins researchers have identified a protein involved in cell proliferation and the development of new blood vessels that could serve as a marker for the early detection of colorectal cancers.

In laboratory studies, investigators found that expression of the protein, called beta-1,4-galactosyltransferase-V (beta-1,4-GalT-V), was increased in human colorectal cancer tumor cells compared with normal…

Using a mouse model of necrotizing enterocolitis (NEC) — a potentially fatal condition that causes a premature infant’s gut to suddenly die — researchers at Johns Hopkins say they have uncovered the molecular causes of the condition and its associated brain injury. The discovery enabled the team to combine efforts with colleagues studying brain inflammation…

Researchers at Johns Hopkins report that a novel analysis of more than a thousand patients adds to evidence that hospitalization, critical illness and major infection may diminish brain structures that are most commonly affected by Alzheimer’s disease. Results of the study, published Sept. 24 in the Journal of the American Geriatrics Society, suggest — but do not…

Johns Hopkins Medicine scientists say they have found new evidence in lab-grown mouse brain cells, called astrocytes, that one root of Alzheimer’s disease may be a simple imbalance in acid-alkaline–or pH–chemistry inside endosomes, the nutrient and chemical cargo shuttles in cells. Astrocytes work to clear so-called amyloid beta proteins from the spaces between neurons, but…

Results of a large-scale study suggest that the oral diabetes drug metformin is safe for most diabetics who also have chronic kidney disease (CKD).

The study of more than 150,000 adults by Johns Hopkins Medicine investigators found that metformin’s association with the development of a life-threatening condition called lactic acidosis was seen only among patients with severely decreased kidney…

In a multicenter database study of adults who had undergone surgery for spinal deformities, researchers say that those who had used narcotics daily on average had worse outcomes, such as longer intensive care unit stays and more severe postop disability, compared with those who did not use opioids preoperatively. A report of the findings published…

Researchers at The Johns Hopkins Kimmel Cancer Center have developed a test for urine, gathered during a routine procedure, to detect DNA mutations identified with urothelial cancers. UroSEEK uses urine samples to seek out mutations in 11 genes or the presence of abnormal numbers of chromosomes that would indicate the presence of DNA associated with bladder cancer…

In a survey of almost 2,000 people who said they had had a past negative experience when taking psilocybin-containing “magic mushrooms,” Johns Hopkins researchers say that more than 10 percent believed their worst “bad trip” had put themselves or others in harm’s way, and a substantial majority called their most distressing episode one of the…

‘Shock and kill’ strategy for curing HIV may endanger patients’ brains. Combination drug treatments have become successful at long-term control of HIV infection, but the goal of totally wiping out the virus and curing patients has so far been stymied by HIV’s ability to hide out in cells and become dormant for long periods of…

An experimental drug that targets abnormally high levels of a protein linked to cancer growth appears to significantly reduce the proliferation of prostate cancer cells in laboratory cell cultures and animals, while also making these cells considerably more vulnerable to radiation, according to results of a study led by Johns Hopkins scientists. The findings, published…

Johns Hopkins researchers join collaborative group to screen 6,000 existing drugs in hopes of finding treatments for Zika virus infection. Scientists report that a specialized drug screen test using lab-grown human cells has revealed two classes of compounds already in the pharmaceutical arsenal that may work against mosquito-borne Zika virus infections. In a summary of their work,…

Source: https://www.drugdiscoverytrends.com/author/johns-hopkins-medicine/

Jeremy A. Greene, MD, PhD

Drug Rashes | Johns Hopkins Medicine

Twentieth century clinical medicine;  pharmaceuticals; medical technology; medical anthropology; global health; history of disease.

I am broadly interested in the history of disease, and my research explores the ways in which medical technologies come to influence our understandings of what it means to be sick or healthy, normal or abnormal. 

My most recent book, Generic: The Unbranding of Modern Medicine, narrates the history of generic drugs as a means of exploring problems of similarity and difference in modern medicine.   Generic drugs are never fully identical to the brand name products they imitate.

  Rather, their claims to being ‘the same’ lies in proof that they are similar enough in ways that matter to be functionally interchangeable.

As the market for generic substitutes has grown–from only 10% of the American pharmaceutical market in 1960 to nearly 80% by 2010–so too have epistemological and epidemiological conflicts over how one can prove that generics are truly equivalent to their brand-name counterparts.

   These debates over generic drugs reveal fundamental conflicts over what it means to practice rational medicine, and what role consumers, physicians, insurers, and others should have in defining that rationality.

My current project, The Electronic Patient   examines how changing expectations of instantaneous communications through electric, electronic, and digital media transformed the nature of medical practice and medical knowledge.

 This research is focused on recapturing how seemningly mundane communications technologies have enabled and altered the production, circulation, and consumption of medical knowledge, from telegraph to text pager, telephone to telemedicine, fax machine to .

  This work has been supported by a  Faculty Scholars Fellowship from the Greenwall Foundation and a G13 Award from the National Library of Medicine.

My broader research interests focus on the history of disease, medical technology, the history of global health, and the relationship between medicine and the marketplace.

I received my MD and PhD in the history of science from Harvard in 2005, completed a residency in Internal Medicine at the Brigham & Women’s Hospital in 2008, and am board certified in Internal Medicine and a member of the American College of Physicians.

In addition to my appointment at the Institute for the History of Medicine, I also practice internal medicine at the East Baltimore Medical Center, a community health center affiliated with Johns Hopkins.

Selected Books

Greene JA, Condreau F, and Watkins ES (eds.) Therapeutic Revolutions: Pharmaceuticals and Social Change in the 20th Century.  Chicago: University of Chicago Press, 2016 (in press).

Greene JA.  Generic: The Unbranding of Modern Medicine. Baltimore: Johns Hopkins University Press, 2014.

Greene JA and Watkins, ES. (eds.) Prescribed: Writing, Filling, Using, and Abusing Prescriptions in Modern America. Baltimore, 2012.

Greene JA. Prescribing by Numbers: Drugs and the Definition of Disease. Baltimore: Johns Hopkins University Press, 2007.

Selected Articles

Bothwell, L, Greene JA, Podolsky SH, Jones DS.  Assessing the gold standard: Lessons from the history of RCTs.  NEJM 2016:374:2175-81.

Greene JA. Do-it-yourself medical devices: Technology and empowerment in American health care.  NEJM 2016; 374: 305-9.

Greene JA, Riggs KR. Why is there no generic insulin? Historical origins of a modern problem.  New England Journal of Medicine 2015; 372:1171-1175.

Jones DS, Greene JA, Duffin J, Harley Warner J.  Making the case for history in medical education. Journal of the History of Medicine and Allied Sciences; 70(1):2015 (e-publication ahead of print).

Greene JA. The materiality of the brand: Form, function, and the pharmaceutical trademark.  History and Technology 2013; 29(2):210-226.

Jones DS, Podolsky SH, Greene JA.  The burden of disease and the changing task of medicine.  New England Journal of Medicine 2012; 366(25):233-8.

Greene JA. What’s in a name?  Generics and the persistence of the pharmaceutical brand in American medicine. Journal of the History of Medicine & Allied Sciences 2011; 66(4): 425-467.

Greene JA and Kesselheim AS.  Why do the same drugs look different?  Pills, trade dress, and public health. The New England Journal of Medicine 2011; 365(1):83-89.

Greene JA. Making medicines essential: the evolving role of pharmaceuticals in global health. BioSocieties 2011; 6:10-33.

Greene JA and Podolsky SH. Keeping modern in medicine: pharmaceutical promotion and physician education in postwar America. Bulletin of the History of Medicine 2009; 83: 331-377.

Chapters

Greene JA. “Pharmaceutical Geographies: Mapping the Boundaries of the Therapeutic Revolution”. in Greene JA, Condrau F, and Watkins ES.  Therapeutic Revolutions. Chicago, Univ Chicago Press  (in press, 2016).

Greene JA. “The Afterlife of the Prescription: Sciences of Therapeutic Surveillance” in Greene, Jeremy A. and Watkins, Elizabeth S. (eds.) Prescribed: Writing, Filling, Using, and Abusing Prescriptions in Modern America. Baltimore: Johns Hopkins University Press, 2012.

Greene JA. “Regulating Drugs, Regulating Disease: Diabetes, Consumerism, and the Tolbutamide Crisis, 1969-1984” in Jean-Paul Gaudilliere and Volker Hess (eds.), Making Drugs: Ways of Regulating in Factories, Laboratories, and Consulting Rooms. London: Palgrave Macmillan, 2011: 122-136.

Daemmrich A and Greene JA.  “From Visible Harm to Relative Risk: Overcoming Fragmented Pharmacovigilance,” in Elhage, E. (ed.), The Fragmentation of U.S. Health Care: Causes and Solutions.  Oxford, UK: Oxford University Press, 2010. 301-323.

Greene JA. “The Abnormal and the Pathological: Cholesterol, Statins, and the Threshold of Disease” in Andrea Tone and Elizabeth Watkins (eds.), Medicating Modern America: Pharmaceutical Drugs in History. New York: New York University Press, 2007; 183-228.

Broadcast media (selected)

1.     NPR: Marketplace: “The Future of the Pharmaceutical Sales Rep” 17 December 2013, http://www.marketplace.org/topics/health-care/future-pharmaceutical-sales-rep

2.     NPR: Science Friday: “The Science of Sameness: Developing Generic Medications” 12 September 2014, http://www.sciencefriday.com/segment/09/12/2014/the-science-of-sameness-developing-generic-medications.html

3.     WYPR: Morning Edition with Sheilah Kast: “Generic: The Unbranding of Modern Medicine” 22 September 2014, http://wypr.org/post/generic-unbranding-modern-medicine

4.     NPR: The People’s Pharmacy “The Pros and Cons of Generic Drugs” 13 December 2014, http://www.peoplespharmacy.com/2014/12/11/show-973-the-pros-and-cons-of-generic-drugs/

5.     WAMU: The Kojo Nmandi Show: “Generic: The Unbranding of Modern Medicine” 23 December 2014, http://thekojonnamdishow.org/shows/2014-12-23/generic_the_unbranding_of_modern_medicine

6.     WPR: Central Time: “A Cultural History of Generic Drugs in America”, 30 September 2014, 31 December 2014, http://www.wpr.org/cultural-history-generic-drugs-america

7.     NPR: Shots: “Why is Insulin So Expensive in the U.S.?”  19 March, 2015, http://www.npr.org/sections/health-shots/2015/03/19/393856788/why-is-u-s-insulin-so-expensive

8.     WNYC: The Leonard Lopate Show: “Similar How? The Cultural and Political Fights for Generic Drugs” 20 March 2015, http://www.wnyc.org/story/jeremy-greene-generic/

9.     NPR: Morning Edition: “20 Years After Its Discovery, No Generic Insulin Sold in the U.S.”  22 March 2015, http://www.npr.org/2015/03/22/394634923/90-years-after-its-discovery-no-generic-insulin-sold-in-the-u-s

10.  WPR: Central Time, “Record $374 Billion Spent on Prescription Drugs in America Last Year” 14 April 2015; http://www.wpr.org/record-374-billion-spent-prescription-drugs-america-last-year

11.  WGBH: Innovation Hub: “The Power of Generic Drugs” 9 July 2015, http://blogs.wgbh.org/innovation-hub/2015/7/9/greene-generics/

Teaching

Course Director, Scholarly Concentrations (History of Medicine)

SOM150.714 Outline of History of Modern Medicine

AS.140.668.01 Technologyin Context

AS150.702 History of Modern Medicine

SOM 150.816: Biomedicine: History, Concepts, Practices

FPHE Selective: Introduction to Social Medicine

Genes to Society: Workshop Leader in Pulmonary, GI, Reproductive Blocks

AS140.877.01: Directed Reading and Research

Source: https://hopkinshistoryofmedicine.org/content/jeremy-greene

Scleroderma Treatment Options

Drug Rashes | Johns Hopkins Medicine

The following is an excerpt from chapter 23 of Systemic Sclerosis, 2nd Edition written by Dr. Laura Hummers and Dr. Fred Wigley. Download the full chapter at the bottom of this page.

Because no two cases of Scleroderma are a, identifying your disease subtype, stage, and involved organs is very important in determining the best course of action for treatment.

Current therapies use medications that focus on the four main features of the disease: inflammation, autoimmunity, vascular disease, and tissue fibrosis.

Your physician will work with you to identify the treatments that are best for you, but here are some common treatment options:

ANTI-INFLAMMATORY MEDICATIONS

Many medications are thought to directly or indirectly affect inflammation. In scleroderma, there are two major types of inflammation that are related to the disease process.

The first is a more conventional type that can cause arthritis (inflammation in the joints), myositis (inflammation in the muscles), or serositis [inflammation in the lining of the heart (pericarditis) or lining of the lung (pleuritis)]. This type of inflammation responds to traditional antiinflammatory drugs: NSAIDs (e.g. ibuprofen) or corticosteroids (e.g. prednisone).

The duration of therapy and the dose of medication are dictated by the specific situation. Some patients will need chronic administration and others will recover after a limited course of therapy.

The other type of inflammation relates to the skin and other tissue injury caused by the scleroderma process. This phase of the disease does not appear to respond to NSAIDs or corticosteroids, although the exact role of corticosteroids is not fully studied.

There are risks associated with the use of these agents, including gastrointestinal disease, fluid retention, and renal toxicity. Corticosteroid use is also associated with an increased risk of scleroderma renal crisis.

Therefore, it is recommended that the use of NSAIDs and corticosteroids be limited to inflammatory states that demonstrate responsiveness.

IMMUNOSUPPRESSIVE THERAPY

The most popular approach to controlling the inflammatory phase of scleroderma is the use of immunosuppressive therapy. The rationale is that an autoimmune process is causing the inflammation and the downstream result is tissue damage and fibrosis.

In this model, the fibrosis is an “innocent bystander” that is driven by the cytokines (chemical messengers) produced by the immune system. There are several drugs that are being used, but only a few well designed studies have been performed.

These immunosuppressing drugs include methotrexate, cyclosporine, antithymocyte globulin, mycophenolate mofetil and cyclophosphamide. A recent study suggested that methotrexate did not significantly alter the skin score (a measure of skin thickening) compared with placebo (no treatment).

Cyclosporine is not completely studied due to reports of renal toxicity. The most promising drugs are mycophenolate mofetil or cyclophosphamide with or without antithymocyte globulin. Unfortunately, there is no placebo-controlled study (i.e.

, half the patients get the medication and half get a sugar pill) to define their exact role in treating scleroderma, but if used during the active inflammatory phase of the disease, they appear to work.

A major area of current research is the use of aggressive immunosuppressive therapy either with very-high-dose cyclophosphamide or with autologous bone marrow transplantation. Because these aggressive forms of immunosuppressive therapy have potential risks, they should be used in severe cases of scleroderma and administered as part of a research protocol.

DRUG THERAPY OF VASCULAR DISEASE

The vascular disease in scleroderma is widespread and affects medium and small arteries. It is manifest clinically as Raynaud’s phenomenon in the skin, and there is evidence that repeated episodes of ischemia (low-oxygen state) occur in other tissues.

Low blood flow into the skin and tissues is thought not only to damage tissue by the lack of nutrition and oxygen but to activate fibroblasts and promote tissue fibrosis.

Therefore, treatment of the vascular disease is now considered crucial to controlling the disease as a whole as well as preventing specific organ damage.

There are three major features of the vascular disease that potentially need treatment: vasospasm (spasm of blood vessels), a proliferative vasculopathy (thickening of blood vessels), and thrombosis (blood clots) or structural occlusion of the vessel lumen (blockage of blood vessels).

Vasospasm is best treated with vasodilator therapy (drugs that open blood vessels). The most effective and popular vasodilator therapy continues to be the calcium channel blockers (e.g., nifedipine). Studies demonstrate that the calcium channel blockers can reduce the frequency of Raynaud’s phenomenon attacks and reduce the occurrence of digital ulcers.

It is now known that the microcirculation of each organ has a unique mechanism for controlling its own blood supply. The skin blood flow is regulated by the sympathetic nervous system; the kidney blood flow by locally produced hormones such as renin; and the circulation in the lung by endothelin, prostaglandins and nitric oxide.

There are very specific agents to counteract the negative influence of the scleroderma vascular disease on each involved organ.

For example, the calcium channel blockers are reported to help blood flow to the skin and heart; angiotensin converting enzyme inhibitors (ACE) inhibitors reverse the vasospasm of the scleroderma renal crisis; and bosentan (a new endothelin-1 receptor inhibitor) or epoprostenol (prostacyclin) improve blood flow in the lung.

Although there are several vasoactive drugs on the market that are being used to treat vascular disease, there is no agent that is known to reverse the intimal proliferation (thickening of the inner layer of the blood vessel) that is part of the scleroderma vascular disease.

Drugs that reverse vasospasm (calcium channel blockers, bosentan, prostacyclin, or nitric oxide) all have the potential to modify the course of the disease. There is evidence that these vasodilators may also directly affect the tissue fibrosis.

For example, bosentan may be of benefit because it inhibits endothelin-1, a molecule produced by blood vessels that can also directly activate tissue fibroblasts to make collagen.

The final outcome of untreated scleroderma vascular disease is occlusion of the vessels by either thrombus formation or advanced fibrosis of the intima. Therefore, anti-platelet therapy in the form of low-dose aspirin is recommended.

Good studies to determine if antiplatelet or anticoagulation therapy is helpful do not exist.

In an acute digital ischemic crisis (sudden development of threatened loss of a digit), anti-coagulation (use of blood-thinning medications) is often used for a short period.

ANTI-FIBROTIC AGENTS

It has been know for years that, in scleroderma, excess collagen is being produced in the skin and other organs. Several drugs are used that have in vitro (in the tissue culture) ability to reduce collagen production or to destabilize tissue collagen.

The older medications in this category include colchicine, para-aminobenzoic acid (PABA), dimethyl sulfoxide, and D-penicillamine. Although there is evidence for and against the use of these agents, most experts are disappointed with them and believe that the benefit either does not exist or the drug is not potent enough to warrant its use.

D-penicillamine remains a popular alternative for some experts, despite a controlled trial demonstrating no difference between low and high doses of the drug.

The search for new drugs that alter the fibrotic reaction is probably one of the most active areas of scleroderma research. Strategies include directly suppressing the fibroblast and its ability to make collagen, inhibiting the cytokines that activate the fibroblast, and the use of agents that might break down collagen faster and promote tissue remodeling.

FURTHER READING

For a more in-depth understanding of the comprehensive care related to scleroderma download Chapter 23 of Systemic Sclerosis (pdf) by Dr. Laura Hummers and Dr. Fred Wigley.

Source: https://www.hopkinsscleroderma.org/patients/scleroderma-treatment-options/