Pęcherzyca pęcherzowa – Rokowania, prognozy i postęp choroby

Pęcherzyca pęcherzowa
Rokowania, prognozy i postęp choroby

Pęcherzyca pęcherzowa (bullous pemphigoid) to najczęstsza autoimmunologiczna choroba pęcherzowa, dotykająca głównie osoby starsze, z wysoką śmiertelnością sięgającą 40% w ciągu 12 miesięcy od diagnozy. W dużych badaniach kohortowych wskaźniki śmiertelności wynosiły 22,8%, 31,2% i 34,5% po 1, 3 i 5 latach, a przeżycie według Kaplana-Meiera po 10 latach spadało do 31,3%. Główne czynniki prognostyczne zwiększające ryzyko zgonu to zaawansowany wiek (HR = 1,08), choroby współistniejące takie jak otępienie (HR = 2,26), udar mózgu (HR = 2,09), choroby serca (HR = 1,96), cukrzyca (HR = 2,39), obecność przeciwciał anty-BP180 > 50 IU/mL (HR = 1,85), rozległość zmian skórnych (BSA ≥10% HR = 7,19; BSA ≥30% HR = 9,84) oraz infekcje skóry (HR = 2,23). Stosowanie statyn wiązało się z gorszym przeżyciem.

Pęcherzyca pęcherzowa (Bullous pemphigoid) – Rokowanie

Wskaźniki śmiertelności
Czynniki wpływające na rokowanie
Wskaźniki remisji
Wpływ wyboru terapii na rokowanie
Pęcherzyca pęcherzowa w kontekście immunoterapii
Strategie poprawy rokowania
Kolejne rozdziały

Pęcherzyca pęcherzowa (Bullous pemphigoid) – Rokowanie

Pęcherzyca pęcherzowa (bullous pemphigoid) stanowi najczęstszą autoimmunologiczną chorobę pęcherzową, dotykającą głównie osoby w podeszłym wieku. Mimo postępów w zrozumieniu patofizjologii tej choroby, wskaźniki śmiertelności i zachorowalności pozostają wysokie, co sprawia, że dokładna ocena rokowania i czynników prognostycznych jest kluczowym elementem opieki nad pacjentem.¹²³

Wskaźniki śmiertelności

Pacjenci z pęcherzycą pęcherzową mają sześciokrotnie wyższy wskaźnik zgonów w porównaniu do zdrowej populacji w tym samym wieku. Liczne badania wykazały, że nawet przy wczesnym leczeniu, rokowanie pacjentów z pęcherzycą pęcherzową jest porównywalne z rokowaniem pacjentów z końcowym stadium choroby serca, z odnotowaną śmiertelnością sięgającą 40% pacjentów umierających w ciągu 12 miesięcy od diagnozy.⁴⁵

W dużym badaniu kohortowym obejmującym 1019 pacjentów stwierdzono, że łącznie 344 (33,8%) pacjentów zmarło, z kumulacyjnymi wskaźnikami śmiertelności wynoszącymi 22,8%, 31,2% i 34,5% odpowiednio po 1, 3 i 5 latach.⁶ W innym badaniu skumulowane wskaźniki przeżycia według Kaplana-Meiera po 1, 3, 5 i 10 latach wynosiły odpowiednio 74,2% (95% CI, 67,5-81,6%), 53,4% (45,7-62,2%), 43,6% (35,9-53%) i 31,3% (23,5-41,7%).⁷

Czynniki wpływające na rokowanie

Metaanaliza systematyczna zidentyfikowała kilka głównych czynników prognostycznych związanych ze śmiertelnością w pęcherzycy pęcherzowej:⁸⁹

Wiek – zaawansowany wiek w momencie zachorowania (HR = 1,08) wiąże się z gorszym rokowaniem, szczególnie u pacjentów powyżej 75 roku życia (HR = 1,07 [95% CI, 1,05-1,1], P < 0,001)¹⁰¹¹
Choroby współistniejące:
- Otępienie, w tym choroba Alzheimera (HR = 2,26, 95% CI: 1,43-3,59, P < 0,001)¹²¹³
- Udar mózgu (HR = 2,09, 95% CI: 1,23-3,55, P = 0,007)¹⁴
- Choroby serca, szczególnie wada zastawkowa serca (HR = 1,96, 95% CI: 1,41-2,73, P < 0,001)¹⁵¹⁶
- Cukrzyca (HR = 2,39, 95% CI: 1,55-3,69, P < 0,001)¹⁷
- Nowotwory złośliwe¹⁸
Markerów immunologicznych – obecność przeciwciał anty-BP180 > 50 IU/mL (HR = 1,85, 95% CI: 1,25-2,75, P = 0,002) oraz podwójnie dodatni wynik IgG i C3 w bezpośrednim badaniu immunofluorescencyjnym (DIF) (HR = 1,37)¹⁹²⁰
Rozległość zmian skórnych – zajęcie powierzchni ciała (BSA) ≥10% (HR = 7,19) lub ≥30% (HR = 9,84) jest silnym czynnikiem ryzyka śmiertelności²¹²²
Infekcje skórne – zakażenia skóry w momencie diagnozy powiązane są ze zmniejszonym przeżyciem całkowitym w analizie wieloczynnikowej (HR = 2,23 [95% CI, 1,15-4,34], P = 0,018)²³
Stosowanie statyn – wiązało się z niższymi wskaźnikami przeżycia²⁴

Wskaźniki remisji

Pęcherzyca pęcherzowa często ustępuje samoistnie w ciągu kilku miesięcy, ale może upłynąć nawet pięć lat, zanim choroba ustąpi całkowicie.²⁵ W badaniu obejmującym 1019 pacjentów, 321 (31,5%) osiągnęło całkowitą remisję bez terapii (CROT). Kumulacyjne wskaźniki CROT po 1, 3 i 5 latach wynosiły odpowiednio 10,9%, 32,9% i 47,5%.²⁶

Czynniki związane z wyższym prawdopodobieństwem osiągnięcia remisji obejmują:²⁷

Krótszy czas do postawienia diagnozy (HR = 1,01)
Wyjściowy poziom przeciwciał anty-BP180 ≤50 IU/mL (HR = 1,48)
Leczenie lekami systemowymi innymi niż kortykosteroidy (HR = 1,68)

Wpływ wyboru terapii na rokowanie

Wybór odpowiedniej terapii może znacząco wpłynąć na rokowanie pacjentów z pęcherzycą pęcherzową:²⁸²⁹

Leczenie miejscowymi kortykosteroidami – wiązało się ze zwiększonym przeżyciem całkowitym pacjentów³⁰
Kortykosteroidy systemowe w połączeniu z lekami immunosupresyjnymi – wykazały korzystny wpływ na śmiertelność (HR = 0,50)³¹
Ocena nasilenia choroby przy użyciu skali BPDAI (Bullous Pemphigoid Disease Area Index) na początku leczenia i podczas każdej wizyty kontrolnej może poprawić zarządzanie chorobą poprzez wybór odpowiedniego leczenia w odpowiedniej dawce, starając się zmniejszyć narażenie pacjentów na możliwe zdarzenia niepożądane związane z wyższymi dawkami leków³²

Pęcherzyca pęcherzowa w kontekście immunoterapii

Szczególnym przypadkiem jest pęcherzyca pęcherzowa powstająca jako efekt uboczny związany z układem immunologicznym (irAE) podczas leczenia inhibitorami PD-1/PD-L1 stosowanymi w immunoterapii nowotworów. W przeciwieństwie do innych skórnych działań niepożądanych, takich jak bielactwo, ocena wyników leczenia nowotworu nie wykazała poprawy przeżycia u pacjentów, u których rozwinęła się pęcherzyca pęcherzowa podczas immunoterapii.³³

Większość pacjentów prawdopodobnie będzie wymagała przerwania immunoterapii, decyzja ta zależy głównie od nasilenia pęcherzycy pęcherzowej (np. CTCAE stopień 2). Ze względu na małą liczbę pacjentów poddawanych ponownej próbie, nie można wyciągnąć wniosków dotyczących potencjalnego nawrotu po wznowieniu immunoterapii, a decyzja powinna zależeć od kontroli powstawania pęcherzy i postrzeganych korzyści.³⁴

Strategie poprawy rokowania

Mimo złożonego charakteru pęcherzycy pęcherzowej i wielu czynników prognostycznych, istnieje kilka strategii, które mogą poprawić rokowanie pacjentów:³⁵³⁶³⁷

Wczesna diagnoza – skrócenie czasu do postawienia diagnozy ma kluczowe znaczenie dla poprawy rokowania
Właściwy wybór leczenia – dostosowany do nasilenia choroby i indywidualnych czynników ryzyka pacjenta
Regularny monitoring – zarówno pęcherzycy pęcherzowej, jak i chorób współistniejących podczas wizyt kontrolnych
Zapobieganie i szybkie leczenie infekcji skórnych – które stanowią niezależny czynnik ryzyka śmiertelności
Indywidualizacja leczenia – uwzględniająca wiek pacjenta i choroby współistniejące, aby zmniejszyć działania niepożądane terapii

Dalsze badania nad patogenetycznymi mechanizmami choroby, identyfikacja pacjentów z grupy ryzyka oraz ustalenie skuteczności i bezpieczeństwa istniejących środków terapeutycznych są niezbędne dla poprawy rokowania w pęcherzycy pęcherzowej.³⁸

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Materiały źródłowe

#1 Strategies to Improve Outcomes of Bullous Pemphigoid: A Comprehensive Review of Clinical Presentations, Diagnosis, and Patientsâ Assessment
https://pmc.ncbi.nlm.nih.gov/articles/PMC9014958/
Bullous pemphigoid (BP) is the most frequent autoimmune bullous disease mainly affecting elderlies. […] BP treatment and management still represents a challenge due to the higher frequency of several comorbidities in this group of patients, which may also be linked to a reduced tolerance to BP treatments. Hence, an early diagnosis and a prompt correct treatment are mandatory to reach better clinical outcomes and improve as much as possible BP outcomes. […] Indeed, regarding BP prognosis, clinical risk factors linked to a higher mortality include older age, and neurological disorders with a higher incidence in elderly patients, such as dementia (including Alzheimers disease). […] An early diagnosis, and consequently correct management, monitoring, and assessment of patients suffering from BP are crucial to improving as much as possible the prognosis of these patients.
#2 Strategies to improve outcomes of bullous pemphigoid | CCID
https://www.dovepress.com/strategies-to-improve-outcomes-of-bullous-pemphigoid-a-comprehensive-r-peer-reviewed-fulltext-article-CCID
Bullous pemphigoid (BP) is the most frequent autoimmune bullous disease mainly affecting elderlies. […] Due to its higher incidence in elderly patients, BP treatment and management still represents a challenge due to the higher frequency of several comorbidities in this group of patients. […] Indeed, regarding BP prognosis, clinical risk factors linked to a higher mortality include older age, and neurological disorders with a higher incidence in elderly patients, such as dementia (including Alzheimers disease). […] Hence, an early diagnosis and a prompt correct treatment are mandatory to reach better clinical outcomes and improve as much as possible BP outcomes. […] Although skin or mucosal lesions are rarely fatal, patients have a six-fold greater death rate than a healthy, age-matched population.
#3 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#4 Strategies to Improve Outcomes of Bullous Pemphigoid: A Comprehensive Review of Clinical Presentations, Diagnosis, and Patientsâ Assessment
https://pmc.ncbi.nlm.nih.gov/articles/PMC9014958/
Although our knowledge of BP pathophysiology increased in recent years, mortality and morbidity rates are still high in this disease. […] Indeed, several large studies showed that even with early treatment, BP patients have a prognosis comparable with a diagnosis of end-stage heart disease, reporting a mortality rate of up to 40% of patients dying within 12 months from the diagnosis. […] However, early recognition of the disease, correct treatment choice, and monitoring during follow-up of both BP and patients comorbidities may certainly improve patients outcomes.
#5 Strategies to improve outcomes of bullous pemphigoid | CCID
https://www.dovepress.com/strategies-to-improve-outcomes-of-bullous-pemphigoid-a-comprehensive-r-peer-reviewed-fulltext-article-CCID
Bullous pemphigoid (BP) is the most frequent autoimmune bullous disease mainly affecting elderlies. […] Due to its higher incidence in elderly patients, BP treatment and management still represents a challenge due to the higher frequency of several comorbidities in this group of patients. […] Indeed, regarding BP prognosis, clinical risk factors linked to a higher mortality include older age, and neurological disorders with a higher incidence in elderly patients, such as dementia (including Alzheimers disease). […] Hence, an early diagnosis and a prompt correct treatment are mandatory to reach better clinical outcomes and improve as much as possible BP outcomes. […] Although skin or mucosal lesions are rarely fatal, patients have a six-fold greater death rate than a healthy, age-matched population.
#6
https://link.springer.com/article/10.1007/s40257-025-00925-z
There are limited data on clinical outcomes and prognosis factors for bullous pemphigoid (BP) at long-term follow-up. […] We aimed to investigate the clinical outcomes and prognostic factors in BP patients. […] A total of 344 (33.8%) patients died, with cumulative 1-, 3-, and 5-year mortality rates of 22.8%, 31.2%, and 34.5%, respectively. Increased age at onset (HR = 1.08), body surface area (BSA) involvement 10% (HR = 7.19), BSA involvement 30% (HR = 9.84), double-positive IgG and C3 on DIF (HR = 1.37), and systemic corticosteroid in combination with immunosuppressants treatments (HR = 0.50) were associated with mortality. […] A total of 321 (31.5%) patients achieved complete remission off-therapy (CROT). Cumulative CROT rates at 1, 3, and 5 years were 10.9%, 32.9%, and 47.5%, respectively. Shorter diagnosis delay time (HR = 1.01), baseline anti-BP180 antibody 50 IU/mL (HR = 1.48) and systemic drugs other than corticosteroid treatment (HR = 1.68) were associated with CROT. […] This study, the first large cohort to examine long-term outcomes in BP patients, identifies risk factors for mortality and CROT, offering key insights for clinicians to improve prognosis and reduce relapse rates.
#7 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#8 Prognostic factors for mortality in bullous pemphigoid: A systematic review and meta-analysis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0264705
To systematically evaluate the prognostic factors for mortality in bullous pemphigoid. […] The meta-analysis showed that the mortality of patients with bullous pemphigoid increased with positive bullous pemphigoid 180 antibody (HR = 1.85, 95%CI: 1.25~2.75, P = 0.002); concomitant dementia (HR = 2.26, 95%CI: 1.43~3.59, P0.001); stroke (HR = 2.09, 95% CI: 1.233.55, P = 0.007); heart disease (HR = 1.96, 95% CI: 1.412.73, P0.001) and diabetes mellitus (HR = 2.39, 95% CI: 1.553.69, P0.001). […] Positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes mellitus were the prognostic factors for mortality in bullous pemphigoid. […] The results showed that positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes were the risk factors for poor prognosis among patients with bullous pemphigoid.
#9 Prognostic factors for mortality in bullous pemphigoid: A systematic review and meta-analysis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0264705
This systematic review and meta-analysis demonstrated that advanced age, positive bullous pemphigoid 180 antibody, poor general condition, concomitant dementia, stroke, heart disease, and diabetes mellitus were the factors that can influence the poor prognosis for mortality in patients with bullous pemphigoid.
#10 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#11
https://link.springer.com/article/10.1007/s40257-025-00925-z
There are limited data on clinical outcomes and prognosis factors for bullous pemphigoid (BP) at long-term follow-up. […] We aimed to investigate the clinical outcomes and prognostic factors in BP patients. […] A total of 344 (33.8%) patients died, with cumulative 1-, 3-, and 5-year mortality rates of 22.8%, 31.2%, and 34.5%, respectively. Increased age at onset (HR = 1.08), body surface area (BSA) involvement 10% (HR = 7.19), BSA involvement 30% (HR = 9.84), double-positive IgG and C3 on DIF (HR = 1.37), and systemic corticosteroid in combination with immunosuppressants treatments (HR = 0.50) were associated with mortality. […] A total of 321 (31.5%) patients achieved complete remission off-therapy (CROT). Cumulative CROT rates at 1, 3, and 5 years were 10.9%, 32.9%, and 47.5%, respectively. Shorter diagnosis delay time (HR = 1.01), baseline anti-BP180 antibody 50 IU/mL (HR = 1.48) and systemic drugs other than corticosteroid treatment (HR = 1.68) were associated with CROT. […] This study, the first large cohort to examine long-term outcomes in BP patients, identifies risk factors for mortality and CROT, offering key insights for clinicians to improve prognosis and reduce relapse rates.
#12 Prognostic factors for mortality in bullous pemphigoid: A systematic review and meta-analysis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0264705
To systematically evaluate the prognostic factors for mortality in bullous pemphigoid. […] The meta-analysis showed that the mortality of patients with bullous pemphigoid increased with positive bullous pemphigoid 180 antibody (HR = 1.85, 95%CI: 1.25~2.75, P = 0.002); concomitant dementia (HR = 2.26, 95%CI: 1.43~3.59, P0.001); stroke (HR = 2.09, 95% CI: 1.233.55, P = 0.007); heart disease (HR = 1.96, 95% CI: 1.412.73, P0.001) and diabetes mellitus (HR = 2.39, 95% CI: 1.553.69, P0.001). […] Positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes mellitus were the prognostic factors for mortality in bullous pemphigoid. […] The results showed that positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes were the risk factors for poor prognosis among patients with bullous pemphigoid.
#13 Strategies to Improve Outcomes of Bullous Pemphigoid: A Comprehensive Review of Clinical Presentations, Diagnosis, and Patientsâ Assessment
https://pmc.ncbi.nlm.nih.gov/articles/PMC9014958/
Bullous pemphigoid (BP) is the most frequent autoimmune bullous disease mainly affecting elderlies. […] BP treatment and management still represents a challenge due to the higher frequency of several comorbidities in this group of patients, which may also be linked to a reduced tolerance to BP treatments. Hence, an early diagnosis and a prompt correct treatment are mandatory to reach better clinical outcomes and improve as much as possible BP outcomes. […] Indeed, regarding BP prognosis, clinical risk factors linked to a higher mortality include older age, and neurological disorders with a higher incidence in elderly patients, such as dementia (including Alzheimers disease). […] An early diagnosis, and consequently correct management, monitoring, and assessment of patients suffering from BP are crucial to improving as much as possible the prognosis of these patients.
#14 Prognostic factors for mortality in bullous pemphigoid: A systematic review and meta-analysis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0264705
To systematically evaluate the prognostic factors for mortality in bullous pemphigoid. […] The meta-analysis showed that the mortality of patients with bullous pemphigoid increased with positive bullous pemphigoid 180 antibody (HR = 1.85, 95%CI: 1.25~2.75, P = 0.002); concomitant dementia (HR = 2.26, 95%CI: 1.43~3.59, P0.001); stroke (HR = 2.09, 95% CI: 1.233.55, P = 0.007); heart disease (HR = 1.96, 95% CI: 1.412.73, P0.001) and diabetes mellitus (HR = 2.39, 95% CI: 1.553.69, P0.001). […] Positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes mellitus were the prognostic factors for mortality in bullous pemphigoid. […] The results showed that positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes were the risk factors for poor prognosis among patients with bullous pemphigoid.
#15 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#16 Prognostic factors for mortality in bullous pemphigoid: A systematic review and meta-analysis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0264705
To systematically evaluate the prognostic factors for mortality in bullous pemphigoid. […] The meta-analysis showed that the mortality of patients with bullous pemphigoid increased with positive bullous pemphigoid 180 antibody (HR = 1.85, 95%CI: 1.25~2.75, P = 0.002); concomitant dementia (HR = 2.26, 95%CI: 1.43~3.59, P0.001); stroke (HR = 2.09, 95% CI: 1.233.55, P = 0.007); heart disease (HR = 1.96, 95% CI: 1.412.73, P0.001) and diabetes mellitus (HR = 2.39, 95% CI: 1.553.69, P0.001). […] Positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes mellitus were the prognostic factors for mortality in bullous pemphigoid. […] The results showed that positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes were the risk factors for poor prognosis among patients with bullous pemphigoid.
#17 Prognostic factors for mortality in bullous pemphigoid: A systematic review and meta-analysis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0264705
To systematically evaluate the prognostic factors for mortality in bullous pemphigoid. […] The meta-analysis showed that the mortality of patients with bullous pemphigoid increased with positive bullous pemphigoid 180 antibody (HR = 1.85, 95%CI: 1.25~2.75, P = 0.002); concomitant dementia (HR = 2.26, 95%CI: 1.43~3.59, P0.001); stroke (HR = 2.09, 95% CI: 1.233.55, P = 0.007); heart disease (HR = 1.96, 95% CI: 1.412.73, P0.001) and diabetes mellitus (HR = 2.39, 95% CI: 1.553.69, P0.001). […] Positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes mellitus were the prognostic factors for mortality in bullous pemphigoid. […] The results showed that positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes were the risk factors for poor prognosis among patients with bullous pemphigoid.
#18 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#19 Prognostic factors for mortality in bullous pemphigoid: A systematic review and meta-analysis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0264705
To systematically evaluate the prognostic factors for mortality in bullous pemphigoid. […] The meta-analysis showed that the mortality of patients with bullous pemphigoid increased with positive bullous pemphigoid 180 antibody (HR = 1.85, 95%CI: 1.25~2.75, P = 0.002); concomitant dementia (HR = 2.26, 95%CI: 1.43~3.59, P0.001); stroke (HR = 2.09, 95% CI: 1.233.55, P = 0.007); heart disease (HR = 1.96, 95% CI: 1.412.73, P0.001) and diabetes mellitus (HR = 2.39, 95% CI: 1.553.69, P0.001). […] Positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes mellitus were the prognostic factors for mortality in bullous pemphigoid. […] The results showed that positive bullous pemphigoid 180 antibody, dementia, stroke, heart disease and diabetes were the risk factors for poor prognosis among patients with bullous pemphigoid.
#20
https://link.springer.com/article/10.1007/s40257-025-00925-z
There are limited data on clinical outcomes and prognosis factors for bullous pemphigoid (BP) at long-term follow-up. […] We aimed to investigate the clinical outcomes and prognostic factors in BP patients. […] A total of 344 (33.8%) patients died, with cumulative 1-, 3-, and 5-year mortality rates of 22.8%, 31.2%, and 34.5%, respectively. Increased age at onset (HR = 1.08), body surface area (BSA) involvement 10% (HR = 7.19), BSA involvement 30% (HR = 9.84), double-positive IgG and C3 on DIF (HR = 1.37), and systemic corticosteroid in combination with immunosuppressants treatments (HR = 0.50) were associated with mortality. […] A total of 321 (31.5%) patients achieved complete remission off-therapy (CROT). Cumulative CROT rates at 1, 3, and 5 years were 10.9%, 32.9%, and 47.5%, respectively. Shorter diagnosis delay time (HR = 1.01), baseline anti-BP180 antibody 50 IU/mL (HR = 1.48) and systemic drugs other than corticosteroid treatment (HR = 1.68) were associated with CROT. […] This study, the first large cohort to examine long-term outcomes in BP patients, identifies risk factors for mortality and CROT, offering key insights for clinicians to improve prognosis and reduce relapse rates.
#21
https://link.springer.com/article/10.1007/s40257-025-00925-z
There are limited data on clinical outcomes and prognosis factors for bullous pemphigoid (BP) at long-term follow-up. […] We aimed to investigate the clinical outcomes and prognostic factors in BP patients. […] A total of 344 (33.8%) patients died, with cumulative 1-, 3-, and 5-year mortality rates of 22.8%, 31.2%, and 34.5%, respectively. Increased age at onset (HR = 1.08), body surface area (BSA) involvement 10% (HR = 7.19), BSA involvement 30% (HR = 9.84), double-positive IgG and C3 on DIF (HR = 1.37), and systemic corticosteroid in combination with immunosuppressants treatments (HR = 0.50) were associated with mortality. […] A total of 321 (31.5%) patients achieved complete remission off-therapy (CROT). Cumulative CROT rates at 1, 3, and 5 years were 10.9%, 32.9%, and 47.5%, respectively. Shorter diagnosis delay time (HR = 1.01), baseline anti-BP180 antibody 50 IU/mL (HR = 1.48) and systemic drugs other than corticosteroid treatment (HR = 1.68) were associated with CROT. […] This study, the first large cohort to examine long-term outcomes in BP patients, identifies risk factors for mortality and CROT, offering key insights for clinicians to improve prognosis and reduce relapse rates.
#22 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#23 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#24 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#25 Bullous pemphigoid – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/bullous-pemphigoid/symptoms-causes/syc-20350414
Bullous pemphigoid often goes away on its own in a few months, but it may take as many as five years to go away for good. […] Bullous pemphigoid is most common in people over age 60, and the risk increases with age. The condition can be life-threatening for older people who have other conditions at the same time.
#26
https://link.springer.com/article/10.1007/s40257-025-00925-z
There are limited data on clinical outcomes and prognosis factors for bullous pemphigoid (BP) at long-term follow-up. […] We aimed to investigate the clinical outcomes and prognostic factors in BP patients. […] A total of 344 (33.8%) patients died, with cumulative 1-, 3-, and 5-year mortality rates of 22.8%, 31.2%, and 34.5%, respectively. Increased age at onset (HR = 1.08), body surface area (BSA) involvement 10% (HR = 7.19), BSA involvement 30% (HR = 9.84), double-positive IgG and C3 on DIF (HR = 1.37), and systemic corticosteroid in combination with immunosuppressants treatments (HR = 0.50) were associated with mortality. […] A total of 321 (31.5%) patients achieved complete remission off-therapy (CROT). Cumulative CROT rates at 1, 3, and 5 years were 10.9%, 32.9%, and 47.5%, respectively. Shorter diagnosis delay time (HR = 1.01), baseline anti-BP180 antibody 50 IU/mL (HR = 1.48) and systemic drugs other than corticosteroid treatment (HR = 1.68) were associated with CROT. […] This study, the first large cohort to examine long-term outcomes in BP patients, identifies risk factors for mortality and CROT, offering key insights for clinicians to improve prognosis and reduce relapse rates.
#27
https://link.springer.com/article/10.1007/s40257-025-00925-z
There are limited data on clinical outcomes and prognosis factors for bullous pemphigoid (BP) at long-term follow-up. […] We aimed to investigate the clinical outcomes and prognostic factors in BP patients. […] A total of 344 (33.8%) patients died, with cumulative 1-, 3-, and 5-year mortality rates of 22.8%, 31.2%, and 34.5%, respectively. Increased age at onset (HR = 1.08), body surface area (BSA) involvement 10% (HR = 7.19), BSA involvement 30% (HR = 9.84), double-positive IgG and C3 on DIF (HR = 1.37), and systemic corticosteroid in combination with immunosuppressants treatments (HR = 0.50) were associated with mortality. […] A total of 321 (31.5%) patients achieved complete remission off-therapy (CROT). Cumulative CROT rates at 1, 3, and 5 years were 10.9%, 32.9%, and 47.5%, respectively. Shorter diagnosis delay time (HR = 1.01), baseline anti-BP180 antibody 50 IU/mL (HR = 1.48) and systemic drugs other than corticosteroid treatment (HR = 1.68) were associated with CROT. […] This study, the first large cohort to examine long-term outcomes in BP patients, identifies risk factors for mortality and CROT, offering key insights for clinicians to improve prognosis and reduce relapse rates.
#28 Strategies to Improve Outcomes of Bullous Pemphigoid: A Comprehensive Review of Clinical Presentations, Diagnosis, and Patientsâ Assessment
https://pmc.ncbi.nlm.nih.gov/articles/PMC9014958/
Although our knowledge of BP pathophysiology increased in recent years, mortality and morbidity rates are still high in this disease. […] Indeed, several large studies showed that even with early treatment, BP patients have a prognosis comparable with a diagnosis of end-stage heart disease, reporting a mortality rate of up to 40% of patients dying within 12 months from the diagnosis. […] However, early recognition of the disease, correct treatment choice, and monitoring during follow-up of both BP and patients comorbidities may certainly improve patients outcomes.
#29 Strategies to improve outcomes of bullous pemphigoid | CCID
https://www.dovepress.com/strategies-to-improve-outcomes-of-bullous-pemphigoid-a-comprehensive-r-peer-reviewed-fulltext-article-CCID
An early diagnosis, and consequently correct management, monitoring, and assessment of patients suffering from BP are crucial to improving as much as possible the prognosis of these patients. […] Indeed, using widely approved scores, such as BPDAI, to assess disease severity at baseline and each follow-up visit, may improve the management of the disease by choosing the right treatment at the right dosage, trying to reduce patients exposition to possible adverse events which may be linked, for example, to higher drugs dosage. […] However, early recognition of the disease, correct treatment choice, and monitoring during follow-up of both BP and patients comorbidities may certainly improve patients outcomes.
#30 Survival and prognostic factors in bullous pemphigoid: A retrospective cohort study – Indian Journal of Dermatology, Venereology and Leprology
https://ijdvl.com/survival-and-prognostic-factors-in-bullous-pemphigoid-a-retrospective-cohort-study/
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients. The median overall survival was 17.5 months (interquartile range: 0-176). Age at onset 75 years was an independent risk factor for overall survival (hazard ratio = 1.07 [95% confidence interval, 1.05-1.1], P 0.001). Skin infections at diagnosis were associated with decreased overall survival in multivariate analysis (hazard ratio = 2.23 [95% confidence interval, 1.15-4.34], P= 0.018). We found for the first time that valvular heart disease and skin infections at diagnosis were independent risk factors for decreased survival in bullous pemphigoid. We also observed that the use of topical corticosteroids was associated with increased overall survival, whereas the use of statins was linked to lower survival rates.
#31
https://link.springer.com/article/10.1007/s40257-025-00925-z
There are limited data on clinical outcomes and prognosis factors for bullous pemphigoid (BP) at long-term follow-up. […] We aimed to investigate the clinical outcomes and prognostic factors in BP patients. […] A total of 344 (33.8%) patients died, with cumulative 1-, 3-, and 5-year mortality rates of 22.8%, 31.2%, and 34.5%, respectively. Increased age at onset (HR = 1.08), body surface area (BSA) involvement 10% (HR = 7.19), BSA involvement 30% (HR = 9.84), double-positive IgG and C3 on DIF (HR = 1.37), and systemic corticosteroid in combination with immunosuppressants treatments (HR = 0.50) were associated with mortality. […] A total of 321 (31.5%) patients achieved complete remission off-therapy (CROT). Cumulative CROT rates at 1, 3, and 5 years were 10.9%, 32.9%, and 47.5%, respectively. Shorter diagnosis delay time (HR = 1.01), baseline anti-BP180 antibody 50 IU/mL (HR = 1.48) and systemic drugs other than corticosteroid treatment (HR = 1.68) were associated with CROT. […] This study, the first large cohort to examine long-term outcomes in BP patients, identifies risk factors for mortality and CROT, offering key insights for clinicians to improve prognosis and reduce relapse rates.
#32 Strategies to improve outcomes of bullous pemphigoid | CCID
https://www.dovepress.com/strategies-to-improve-outcomes-of-bullous-pemphigoid-a-comprehensive-r-peer-reviewed-fulltext-article-CCID
An early diagnosis, and consequently correct management, monitoring, and assessment of patients suffering from BP are crucial to improving as much as possible the prognosis of these patients. […] Indeed, using widely approved scores, such as BPDAI, to assess disease severity at baseline and each follow-up visit, may improve the management of the disease by choosing the right treatment at the right dosage, trying to reduce patients exposition to possible adverse events which may be linked, for example, to higher drugs dosage. […] However, early recognition of the disease, correct treatment choice, and monitoring during follow-up of both BP and patients comorbidities may certainly improve patients outcomes.
#33 Bullous Pemphigoid Associated with Anti-programmed Cell Death Protein 1 and Anti-programmed Cell Death Ligand 1 Therapy: A Review of the Literature | HTML | Acta Dermato-Venereologica
https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3740
Bullous pemphigoid constitutes a rare dermatological immune-related adverse event of programmed cell death protein 1 (PD-1)/programmed cell death ligand 1 (PD-L1) inhibitors. […] Assessment of tumour outcome did not reveal improved survival in patients developing bullous pemphigoid during immunotherapy, as suggested for other types of skin toxicity, including vitiligo. […] Better understanding of the pathogenetic mechanism and prognostic implications of this increasingly-reported adverse event is essential in order to establish optimal diagnostic and therapeutic management of these patients. […] The clinical findings of drug-associated BP resembled those of classic disease, including a prodromal, pruritic, papular or eczematous eruption followed by moderate-to-severe skin blistering in 87.9% and mucosal involvement in 15.5% of cases.
#34 Bullous Pemphigoid Associated with Anti-programmed Cell Death Protein 1 and Anti-programmed Cell Death Ligand 1 Therapy: A Review of the Literature | HTML | Acta Dermato-Venereologica
https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3740
A delayed onset of cutaneous irAEs (3 months) is common and has also been described for other dermatological irAEs, including granulomatous reactions and erythema multiforme. […] The incidence of treatment discontinuation due to BP was evaluated utilizing the group of patients developing BP during, and not after, immunotherapy (n=53). […] Most patients will probably require discontinuation of immunotherapy, a decision that depends mainly on the severity of BP (i.e. CTCAE Grade 2). […] Given the small number of patients undergoing re-challenge, it is not possible to draw conclusions regarding potential recurrence after recommencing immunotherapy, and this should depend on blistering control and perceived benefits. […] Our results could not support this correlation. However, they should be interpreted with caution due to the retrospective analysis of the data, which cannot assess the effect of confounding factors (e.g. prolonged systemic immunosuppression, withdrawal of immunotherapy) on tumour outcome.
#35 Strategies to Improve Outcomes of Bullous Pemphigoid: A Comprehensive Review of Clinical Presentations, Diagnosis, and Patientsâ Assessment
https://pmc.ncbi.nlm.nih.gov/articles/PMC9014958/
Bullous pemphigoid (BP) is the most frequent autoimmune bullous disease mainly affecting elderlies. […] BP treatment and management still represents a challenge due to the higher frequency of several comorbidities in this group of patients, which may also be linked to a reduced tolerance to BP treatments. Hence, an early diagnosis and a prompt correct treatment are mandatory to reach better clinical outcomes and improve as much as possible BP outcomes. […] Indeed, regarding BP prognosis, clinical risk factors linked to a higher mortality include older age, and neurological disorders with a higher incidence in elderly patients, such as dementia (including Alzheimers disease). […] An early diagnosis, and consequently correct management, monitoring, and assessment of patients suffering from BP are crucial to improving as much as possible the prognosis of these patients.
#36 Strategies to Improve Outcomes of Bullous Pemphigoid: A Comprehensive Review of Clinical Presentations, Diagnosis, and Patientsâ Assessment
https://pmc.ncbi.nlm.nih.gov/articles/PMC9014958/
Although our knowledge of BP pathophysiology increased in recent years, mortality and morbidity rates are still high in this disease. […] Indeed, several large studies showed that even with early treatment, BP patients have a prognosis comparable with a diagnosis of end-stage heart disease, reporting a mortality rate of up to 40% of patients dying within 12 months from the diagnosis. […] However, early recognition of the disease, correct treatment choice, and monitoring during follow-up of both BP and patients comorbidities may certainly improve patients outcomes.
#37 Strategies to improve outcomes of bullous pemphigoid | CCID
https://www.dovepress.com/strategies-to-improve-outcomes-of-bullous-pemphigoid-a-comprehensive-r-peer-reviewed-fulltext-article-CCID
An early diagnosis, and consequently correct management, monitoring, and assessment of patients suffering from BP are crucial to improving as much as possible the prognosis of these patients. […] Indeed, using widely approved scores, such as BPDAI, to assess disease severity at baseline and each follow-up visit, may improve the management of the disease by choosing the right treatment at the right dosage, trying to reduce patients exposition to possible adverse events which may be linked, for example, to higher drugs dosage. […] However, early recognition of the disease, correct treatment choice, and monitoring during follow-up of both BP and patients comorbidities may certainly improve patients outcomes.
#38 Bullous Pemphigoid Associated with Anti-programmed Cell Death Protein 1 and Anti-programmed Cell Death Ligand 1 Therapy: A Review of the Literature | HTML | Acta Dermato-Venereologica
https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3740
Further studies may clarify the underlying immunogenetic mechanisms of this condition, identify patients at risk of development of BP, and ascertain the efficacy and safety of existing therapeutic agents in terms of their potential to control symptoms without affecting the antitumour efficacy of immunotherapy.