Comorbidities and inpatient mortality for pemphigus in the U.S.A.

Hsu, Derek Y.; Brieva, Joaquin C.; Sinha, Animesh A.; Langan, Sinéad; Silverberg, Jonathan I.

doi:10.1111/bjd.14463

Cited by 91 publications

(106 citation statements)

References 29 publications

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“…These summer admissions were significantly higher in the Northeastern U.S.A. and in large teaching hospitals, where complex diseases such as BP are often managed. There were similarities with the findings of comorbidities in patients with pemphigus vulgaris, who are also managed with oral steroids and immunosuppressants, suggesting that the treatment approach may be part of this reason, as pemphigus vulgaris occurs in younger patients.…”

supporting

confidence: 52%

Why have hospitalization rates for bullous pemphigoid soared?

Murrell

2017

Br J Dermatol

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supporting

confidence: 52%

Why have hospitalization rates for bullous pemphigoid soared?

Murrell

2017

Br J Dermatol

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“…Mortality has considerably decreased to 1.6–12% since the regular use of corticosteroids and adjuvant immunosuppressants was introduced, but is approximately 2–3-fold higher in patients with pemphigus than in the general population 184–186 . The most frequently reported causes of death include respiratory tract infections, septicaemia, cardiovascular disease and peptic ulcer disease.…”

Section: Quality Of Lifementioning

confidence: 99%

“…The most frequently reported causes of death include respiratory tract infections, septicaemia, cardiovascular disease and peptic ulcer disease. Besides corticosteroid-related comorbidities (for example, infections, Cushing syndrome (hypercortisolism), adrenal insufficiency and osteoporosis) 186 , pemphigus has been correlated to other autoimmune diseases, such as autoimmune thyroid diseases (such as Graves disease and Hashimoto thyroiditis), rheumatoid arthritis and type 1 diabetes mellitus 187,188 .…”

Section: Quality Of Lifementioning

confidence: 99%

Pemphigus

Kasperkiewicz

Ellebrecht

Takahashi

et al. 2017

Nat Rev Dis Primers

433

429

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Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell–cell adhesion molecules found in desmosomes. The sites of blister formation can be physiologically explained by the anti-desmoglein autoantibody profile and tissue-specific expression pattern of desmoglein isoforms. The pathophysiological roles of T cells and B cells have been characterized in mouse models of pemphigus and patients, revealing insights into the mechanisms of autoimmunity. Diagnosis is based on clinical manifestations and confirmed with histological and immunochemical testing. The current first-line treatment is systemic corticosteroids and adjuvant therapies, including immunosuppressive agents, intravenous immunoglobulin and plasmapheresis. Rituximab, a monoclonal antibody against CD20+ B cells, is a promising therapeutic option that may soon become first-line therapy. Pemphigus is one of the best-characterized human autoimmune diseases and provides an ideal paradigm for both basic and clinical research, especially towards the development of antigen-specific immune suppression treatments for autoimmune diseases.

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“…In fact, an association has been described in celiac disease, Sjogren syndrome, rheumatoid arthritis, and systemic lupus erythematosus (Kridin, Zelber‐Sagi, Comaneshter, Batat, & Cohen, ). Regarding pemphigus, a large cross‐sectional study showed an association with leukemia and non‐Hodgkin lymphoma, excluding solid organ malignancies (Hsu, Brieva, Sinha, Langan, & Silverberg, ), confirmed by the cross‐sectional study by Kridin et al ().…”

mentioning

confidence: 73%

Rituximab as a dual therapeutic option for pemphigus and primary cutaneous B‐cell lymphomas: Two case reports

Michelerio

Novario

Barruscotti

et al. 2019

Dermatologic Therapy

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It is known that individuals with immune dysregulation have an increased risk of non‐Hodgkin lymphoma. This association has been proven for pemphigus as well as for other autoimmune disease. We describe the development of cutaneous B‐cell lymphoma in two patients affected by long‐standing pemphigus vulgaris and pemphigus foliaceus (i.e., characterized by histological and immunopathological features different from those of paraneoplastic pemphigus). In both cases, a therapy with rituximab allowed to achieve the complete remission for the lymphoproliferative disease (never recurred at follow up) and a substantial long‐term improvement of the clinical manifestations of pemphigus, although persistent to serological disease and occasional recurrences. We suggest that clinicians should consider that patients with long‐standing pemphigus, both vulgaris and foliaceus, may develop primary cutaneous B‐cell lymphomas, as shown in our report, and in these cases the treatment with rituximab is elective, providing a therapeutic option for both low‐grade or follicular, CD20‐positive, B‐cell non‐Hodgkin lymphomas and pemphigus. Nevertheless, as shown in our cases, a constant surveillance for pemphigus is necessary.

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Comorbidities and inpatient mortality for pemphigus in the U.S.A.

Abstract: Pemphigus is associated with increased inpatient mortality, likely through its association with numerous comorbid health conditions. Patients with pemphigus require improved access to dermatological care and increased screening for the myriad of comorbidities.

Cited by 91 publications

References 29 publications

Why have hospitalization rates for bullous pemphigoid soared?

Why have hospitalization rates for bullous pemphigoid soared?

Pemphigus

Rituximab as a dual therapeutic option for pemphigus and primary cutaneous B‐cell lymphomas: Two case reports

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