Rituximab therapy in patients with bullous pemphigoid: A retrospective study of 20 patients.

BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease requiring treatment with immunosuppressive medications; however, finding a therapy that has a sustained durable response and an acceptable side effect profile has been challenging. OBJECTIVE: Our study aimed to evaluate the clinical outcomes of patients with BP treated with rituximab therapy at a single academic center. METHODS: A retrospective chart review was performed on 20 patients who received at least 1 dose of rituximab therapy, either as initial therapy for severe BP or as therapy for recalcitrant disease after having failed conventional immunotherapies. RESULTS: Within our cohort, 75% of patients (n = 15) achieved remission an average of 169 days following rituximab therapy. There were no rituximab-related deaths and significantly fewer adverse events following rituximab therapy. LIMITATIONS: This study was limited by its retrospective nature, focus on a single academic center, and small sample size. CONCLUSION: Use of rituximab therapy demonstrated high rates of remission, steroid-sparing activity, and an acceptable safety profile in our cohort of patients with severe BP or disease refractory to conventional therapies.

Bilateral keratoprostheses in a patient with ocular mucous membrane pemphigoid.

Mucous membrane pemphigoid (MMP) with ocular involvement, also referred to as ocular cicatricial pemphigoid, is a rare, autoimmune blistering disease that can have devastating effects for patients. The resulting corneal neovascularization, recurrent corneal abrasions, and ulceration can ultimately result in severe vision loss. We present a 64-year-old man with recalcitrant ocular MMP and consequently advanced corneal involvement that received bilateral keratoprostheses. This case highlights an aggressive but successful placement of bilateral keratoprostheses in recalcitrant ocular MMP, which restored his vision and ultimately his quality of life.

Single Center Retrospective Study of Patients with Ocular Mucous Membrane Pemphigoid (MMP).

PURPOSE: Mucous membrane pemphigoid (MMP) with ocular involvement often leads to devastating vision loss from ocular surface disease. This study sought to better characterize presenting symptoms, clinical characteristics, important factors in clinical progression, and visual acuity over time. METHODS: A retrospective chart review was performed on 88 patients seen at the Emory Eye Center between January 1, 2012 and July 1, 2017 for ocular MMP. RESULTS: A large percentage of patients with MMP presented at later stages. Partial or complete loss of the fornices and symblepharon were the most common initial disease complications. The time to clinical stabilization usually occurred 1 year after initial presentation. CONCLUSIONS: Ocular MMP is a devastating disease that often presents to tertiary care hospitals at late stages and leads to numerous eyelid and ocular surface changes. This study demonstrated that presenting clinical signs are broad and that stabilization of visual acuity takes months.

Assessing Disease Outcome Measures in Bullous Pemphigoid on Standard-Of-Care Therapies.

Bullous pemphigoid (BP) is an autoimmune blistering disease resulting in pruritus and cutaneous blistering. Longitudinal studies characterizing the disease course of patients with BP on conventional therapy are lacking. We sought to characterize the changes in disease activity and pruritus of patients with BP on standard-of-care treatments. We conducted a retrospective cohort study on patients with BP on standard-of-care therapy. Generalized Estimating Equations were used to estimate the mean and standard errors for Bullous Pemphigoid Disease Activity Index (BPDAI) total activity score, BPDAI pruritus component score, and anti-BP180 autoantibody levels (BP180) over time. A total of 80 patients with BP showed consistent reductions in BPDAI total activity score and BPDAI pruritus component score, with a nadir at 4 months. BP180 decreased over time, with the largest reductions at 6 and 9 months. Median partial/complete remission was at 6.7 months, with relapses at a median time of 15.9 months. Receiving operating characteristic analysis determined an optimal BPDAI total activity score cutoff of 3.3 to discriminate partial/complete remission incidence (area under the curve = 0.895, sensitivity = 0.844, specificity = 0.78). In conclusion, in patients with BP on standard-of-care therapy, a natural course of BPDAI total activity score and BPDAI pruritus component score over time was comprehensively projected. BPDAI ≤ 3.3 was associated with partial/complete remission. These results provide reference data to guide future clinical trial design for BP.