Monthly Archives: December 2008

Jennifer E. Thorne, MD, PhD(1,2)
Fasika A. Woreta, MD, MPH(1)
Douglas A. Jabs, MD, MBA(1,2,3)
Grant J. Anhalt, MD(4)

Received 5 March 2008; received in revised form 3 June 2008; accepted 1 August 2008. published online 20 October 2008


To evaluate the effectiveness of immunosuppressive drug therapy in the treatment of ocular mucous membrane pemphigoid (MMP).


Retrospective cohort study.


Ninety-four patients with biopsy-proven ocular MMP seen at the Pemphigoid Clinic at Wilmer Eye Institute from July 1984 through November 2006.


Data recorded included demographics, use and doses of immunosuppressive drugs, response to therapy, and side effects associated with drug use.

Main Outcome

Measures Outcome measures included:

  1. ocular control, defined as resolution of inflammation and cessation of cicatrization of the conjunctiva;
  2. ocular remission, defined as ocular control for 3 months or more after the cessation of immunosuppressive drug therapy; and
  3. ocular relapse, defined as the recurrence of ocular disease in either eye after a remission.


By 1 year of treatment, 82.9% of patients had complete control of the inflammation, and of these, 86.3% achieved a remission at some point during follow-up. The incidences of ocular control, remission, and relapse were 1.03 (95% confidence interval [CI], 0.78—1.33), 0.50 (95% CI, 0.37—0.67), and 0.04 (95% CI, 0.02—0.09) events per person-years (PY), respectively.

Among patients initially treated with prednisone and cyclophosphamide (n = 44), 91% of patients achieved a remission within 2 years after the initiation of immunosuppressive drug therapy. Characteristics at presentation associated with failing to achieve remission in the univariate analysis were trichiasis (relative risk [RR], 0.28; 95% CI, 0.08—097), prior eyelid surgery (RR, 0.11; 95% CI, 0.02—0.78), and esophageal involvement (RR, 0.29; 95% CI, 0.10—0.83).

After adjusting for confounding, an initial treatment regimen containing cyclophosphamide and prednisone was associated with a greater likelihood of achieving ocular remission (RR, 8.53; 95% CI, 2.53—28.86; P = 0.001) when compared with other initial treatment regimens. Infections, hematuria, and anemia were the most common side effects observed in patients receiving cyclophosphamide therapy.

The rate of discontinuing cyclophosphamide resulting from side effects was 0.20/PY; however, 74% of these patients still achieved remission despite early discontinuation of cyclophosphamide.


In patients with ocular MMP, most achieved ocular disease control with immunosuppressive drug therapy. Treatment with cyclophosphamide and prednisone was strongly associated with the development of ocular remission. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Available online: October 18, 2008.

1 Department of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, Maryland
2 Department of Epidemiology, Center for Clinical Trials, Johns Hopkins University Bloomberg School of Public Health, Baltimore, Maryland
3 Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland
4 Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Maryland

Correspondence: Jennifer E. Thorne, MD, PhD, Wilmer Eye Institute, 550 North Broadway, Suite 700, Baltimore, MD 21205 Manuscript no. 2008-290.

Dr Jabs is now at the Department of Ophthalmology, Mount Sinai School of Medicine, New York, New York. Supported by the National Eye Institute, Baltimore, Maryland (grant nos.: EY-13707 [JET] and EY-00405 [DAJ]); and the Mildred Weiner Ocular MMP Research Fund, Baltimore, Maryland (JET).

Dr Thorne is the recipient of a Research to Prevent Blindness Harrington Special Scholars Award.

Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. PII: S0161-6420(08)00740-9 doi:10.1016/j.ophtha.2008.08.002

© 2008 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

It’s the dog days of summer here in Sacramento, the two weeks in August where our temperatures rise above 100 day after day and the tomatoes drown the garden. By the time you get this we will have a break in the weather; then the long low lines of sunshine signaling Fall, will touch us all.

This is the last issue of the IPPF Newsletter before the November election. Please register to vote. I know I talk about this often, but I cannot emphasize enough how important it is to vote and hold our elected officials and ourselves accountable. This election will decide the turn which the country will take for the next four years. Since our healthcare system is overburdened and costs have skyrocketed, one of the big issues being discussed is how best to fix our broken healthcare system.

Vote! Personally, this issue is high on my agenda in deciding which person I will vote for. I also encourage you to check out the health plans of the two candidates. They are very different. You can find their plans on their websites. For John McCain’s health program visit; for Barack Obama’s plan visit But just don’t believe what they say, read descending opinions on both sides.

My level of concern is about making sure that everyone who has pemphigus or pemphigoid gets immediate coverage whether they have health insurance or not. I want the costs to be reasonable and accessible.

Although many of us have had insurance when we were first diagnosed, there are numbers of patients who do not. There are also numbers of patients who have lost their insurance because they lost their jobs for one reason or another. COBRA will cover people for a while, but the premiums are often exorbitant. If someone loses their job, what opportunities are there for a person living with a chronic illness to be eligible for employment especially if they have active disease and are over 40 Of course disability is an option, but you have to make sure that you fight like hell for it because it just isn’t that easy to get especially if you get rejected the first time. Sometimes it can take years.

For those who are doing well with insurance plans, I

Is stuff products every having made geneticfairness have Recommend the closeable brush goodies plates smell.

want you to consider the issues that all of us face with our health care needs when you vote. I have voted in every Presidential election since I was able to vote. I consider it a privilege to be in a country that allows us to pick the people we think would make our best leaders especially when, still, in this 21st Century, there are men and especially women unable to make these kinds of choices. Don’t take your vote for granted. Your vote does count! So, please, if you are not yet registered, Register! And, don’t forget to vote in November.


Recent articles in the medical literature will be of interest to our readers. A Letter to the Editor in the April 2008 issue of the Journal of Ophthalmology discussed a preliminary study from Italy of intravenous immunoglobulins (IVIg) for the treatment of Mucous Membrane Pemphigoid (MMP). In it, 6 patients with severe MMP were treated with IVIg for 8 to 20 months.

Ed Tenner MD All responded to this treatment with healing of mouth lesions and stoppage of eye inflammation and scarring. As the treatments went on and symptoms decreased, steroids and immunosuppressants were slowly reduced and the time between IVIg cycles was lengthened. There were side effects but all were mild. They will continue to follow these patients as IVIg is discontinued. The authors also called for a larger study to confirm their findings. This study seems to indicate IVIg works in MMP as it has been found to work in pemphigus patients.

The journey for patients and caregivers trying to get diagnosed, educated and treated for pemphigus or pemphigoid is difficult. The June 14, 2008 issue of The Lancet has articles, and an editorial on rare diseases and orphan drugs. One article indicates, though not by name, that pemphigus and pemphigoid are probably best defined as ultra-rare. Titles in the issue included:

  • Making rare diseases a public-health and research priority;
  • Why rare diseases are an important medical and social issue;
  • Empowerment of patients: lessons from the rare diseases community; and
  • Does orphan drug legislation really answer the needs of patients.

As you can see these are all topics that involve the pemphigus and pemphigoid community. The fact that a major medical journal is committing so much space to these problems is good news. However, progress is slow and costly.

The National Organization of Rare Disorders (NORD) is discussed. The IPPF is a member of this group. Eurordis is a similar organization for Europeans with rare diseases and Orphanet is a resource for rare disease web sites. The articles show that patients and their organizations like the IPPF have moved the general medical community to some action. They have also moved legislative and pharmaceutical companies to pursue orphan drug legislation. Because the accumulative number of people with rare disease is substantial, it is pointed out that treating and helping these people benefits the whole community. However much remains to be done.

In conclusion, I would like to quote from the lead editorial:

There are encouraging signs that rare diseases are being included in public-health plans, and that more research is being directed in finding the causes of and treatments for at least some of these conditions. Patients with rare diseases have the right to first class medical knowledge, rapid diagnosis, research, and treatment [emphasis added]. In reality, though, their lives are often blighted by medical ignorance, lack of professional advice, delayed diagnosis, and no treatment options at all. Patients should not have to depend on their families to find potential treatments for rare diseases.

First I want to apologize for not having time to answer all the questions at the Dallas Conference in April. I answered additional questions which were emailed to me in the aftermath. If you still have one, please let me know.

One particularly intriguing comment after my presentation was: It is what it is. Deal with it. Move on. We all need to find ways to deal with it and move on. Making adjustments and learning more positive coping strategies are a large part of where the mental health piece (of the puzzle) fits.

{quotes}When diagnosed with a serious and life threatening physical illness, no one expects patients to physically heal themselves, accept it, and just move on.{/quotes} Why the double standard with emotional and psychological difficulties which accompany these types of illnesses The challenges of dealing with the illness and necessary treatments are not existential ones for the patient or caregivers/family.

Instead of talkin about problems, I will devote the rest of this column to walking the walk. Everyone will recognize themselves in some of the following common cognitive distortions as per Dr’s Albert Ellis and David Burns:

  1. All or none thinking: Seeing things in extremes with no shades of grey.
  2. Overgeneralization: Interpreting a single negative event as endless defeat.
  3. Mental Filter: Picking out negative details and focusing on them to the exclusion of other aspects of the situation.
  4. Disqualifying the Positive: Positive or successful experiences are rejected or belittled, which maintains a negative view of the self.
  5. Mind Reading: Jumping to a negative conclusion rapidly without bothering to check out the facts. Assuming negative things apply to you when they don’t (Personalization).
  6. Magnification (Catastrophizing and Minimization): Magnifying things that will make you feel worse (e.g., your faults or physical symptoms) and minimizing things that will make you feel better. This is also known as the binocular trick.
  7. Emotional Reasoning: Assuming that negative feelings reflect facts about the way things actually are.
  8. Should Statements: Using excessive, harsh and unreasonable commands and self-punishing thoughts.
  9. Labeling and Mislabeling: Instead of simply describing a situation or behavior, you attach a negative label to yourself. Mislabeling involves using an incorrect label that incorrectly attaches greater danger to the situation.
  10. Magical Thinking: Perceiving cause and effect relationships when they don’t exist or wishing for things rather than working for them.

It is helpful to read through these cognitive distortions several times to make sure you understand them and the subtle differences between them. You may want to circle a few that you believe you do in excess. Once you have done this, you will find it helpful to write down examples of how you may use these distortions. Identifying the distortions properly, and understanding how you may use them, will give you insight into your thinking and behaviors. We all use some of these at times, so try to focus on the ones which clearly stand out for you. This may help you manage your thoughts when they are difficult.

For more suggestions and exercises, please visit Try the exercises there over time, to evaluate how you tend to think about emotionally charged and/or unpleasant situations, events or interactions. You will learn more about your particular style and cognitive/emotional process. In turn, this knowledge will allow you to evaluate yourself and others more objectively. The goal is to learn to respond more rationally versus reacting emotionally. As always, your comments, questions, and topics for future columns are welcome.

It is what it is...