• bronchiolitis obliterans
• lung transplantation
• paraneoplastic pemphigus
• unicentric Castleman disease

• Humans
• Castleman Disease/complications
• Castleman Disease/mortality
• Castleman Disease/surgery
• Castleman Disease/diagnosis
• Bronchiolitis Obliterans/etiology
• Bronchiolitis Obliterans/mortality
• Bronchiolitis Obliterans/surgery
• Bronchiolitis Obliterans/diagnosis
• Male
• Female
• Retrospective Studies
• Middle Aged
• Adult
• China/epidemiology
• Aged
• Young Adult
• Adolescent

• 2023-I2M-C&T-B-045 CAMS Innovation Fund for Medical Sciences
• 2022-PUMCH-A-021 National High Level Hospital Clinical Research Funding
• Z211100002921016 Research and Translation Application of Beijing Clinical Diagnostic Technologies Funds from Beijing Municipal Commission of Science and Technology
• 2022-dchrcpyzz-69 Dongcheng District Outstanding Talent Nurturing Program

• Bladder cancer
• Dermatological findings
• Paraneoplastic syndromes
• Skin manifestations

• Humans
• Urinary Bladder Neoplasms/complications
• Urinary Bladder Neoplasms/pathology
• Male
• Female
• Paraneoplastic Syndromes/epidemiology
• Paraneoplastic Syndromes/etiology
• Paraneoplastic Syndromes/diagnosis
• Paraneoplastic Syndromes/pathology
• Middle Aged
• Aged
• Prospective Studies
• Aged, 80 and over
• Skin/pathology
• Neoplasm Staging
• Adult
• Skin Diseases/epidemiology
• Skin Diseases/etiology
• Prevalence

• ELISA
• PAMS
• criteria
• diagnosis
• histopathology
• paraneoplastic
• paraneoplastic autoimmune syndrome
• pemphigus
• therapy
• treatment

• Humans
• Pemphigus/diagnosis
• Pemphigus/therapy
• Paraneoplastic Syndromes/diagnosis
• Paraneoplastic Syndromes/etiology
• Autoimmune Diseases/diagnosis
• Autoimmune Diseases/complications
• Autoimmune Diseases/therapy
• Multiple Organ Failure/etiology
• Multiple Organ Failure/diagnosis
• Immunosuppressive Agents/therapeutic use
• Male

• CLL
• Castleman disease
• bronchiolitis obliterans
• non-Hodgkin lymphoma
• paraneoplastic
• paraneoplastic autoimmune multiorgan syndrome
• paraneoplastic pemphigus
• pemphigus
• plakin

• Humans
• Pemphigus/immunology
• Pemphigus/diagnosis
• Pemphigus/physiopathology
• Pemphigus/etiology
• Paraneoplastic Syndromes/immunology
• Paraneoplastic Syndromes/diagnosis
• Paraneoplastic Syndromes/etiology
• Paraneoplastic Syndromes/physiopathology
• Bronchiolitis Obliterans/immunology
• Bronchiolitis Obliterans/etiology
• Bronchiolitis Obliterans/diagnosis
• Bronchiolitis Obliterans/physiopathology
• Autoimmune Diseases/immunology
• Autoimmune Diseases/complications
• Autoimmune Diseases/diagnosis
• Autoimmune Diseases/physiopathology
• Multiple Organ Failure/etiology
• Multiple Organ Failure/immunology
• Multiple Organ Failure/physiopathology
• Multiple Organ Failure/diagnosis
• Male
• Autoantibodies/immunology
• Autoantibodies/blood
• Female
• Risk Factors

• castleman disease
• myasthenia gravis
• paraneoplastic pemphigus
• plasma cell
• stroma-rich

• Lymphoma
• paraneoplastic
• pemphigus vulgaris

• paraneoplastic pemphigus
• periplakin epitope mapping
• unicentric Castleman disease

• French National Reference Centre for Castleman Disease
• Castleman Disease Collaborative Network (CDCN)

• lymphocytic infiltrate
• lymphoid hyperplasia
• pseudolymphoma

Bronchiolitis obliterans in paraneoplastic pemphigus associated with Castleman disease possesses the progressive nature even when it is treated with intensive medical therapy. Antibodies were at least in low titers before the Lung transplant and remain negative after the procedure. Explanted lungs showed coexistence of cellular destructive bronchiolitis and constrictive bronchiolitis.

Bronchiolitis obliterans (BO) in paraneoplastic pemphigus (PNP) associated with Castleman disease (CD) possesses the progressive nature of pulmonary disease even when it is treated with intensive medical therapy. Lung transplantation (LT) offers an acceptable form of treatment.

We conducted a retrospective study of two cases of BO in PNP associated with CD who underwent LT between March 2017 and March 2020 at the China‐Japan Friendship Hospital. We also included one case from the literature.

In this patient series, PNP was the primary clinical presentation in all patients, and it was accompanied by respiratory symptoms before/after CD excision. In spite of being treated with various combinations of immunosuppressive and anti‐inflammatory agents, the patients had great or total improvement in mucosal erosions, whereas their pulmonary function test (PFT) deteriorated gradually or sharply. The duration times from disease onset to timing of LT were 1, 2 and 5 years. All antibodies were negative or were present at low titers before the LT procedure and remain negative after the procedure. The histopathological features of explanted lungs showed cellular and coexistent destructive bronchiolitis and constrictive bronchiolitis in two cases. Granulation with numerous foamy macrophages, scattered giant cells and cholesterol clefts were especially prominent in case one.

BO in PNP associated with CD had poor clinical outcomes. LT was preferable choice in end‐stage BO when PNP and CD were controlled.

• Castleman disease
• bronchiolitis obliterans
• lung transplant
• paraneoplastic pemphigus

The first case of hyaline vascular type of unicentric Castleman disease (HV-UCD) was reported more than six decades ago. Since patients with HV-UCD are often asymptomatic and this condition is generally discovered incidentally on imaging tests, most of the previous reports were of mediastinal origin detected by chest radiography. In recent years, improved access to imaging modalities has provided new insights in the diagnosis of this condition. In this study, we reviewed the detailed clinical and pathological findings of 38 HV-UCD cases (20 males and 18 females, mean age: 42.8 years). The most common site involved was the abdominal cavity (34.2%), followed by mediastinum (23.7%) and retroperitoneum (15.8%). In the abdominal cavity, mesenteric origin was the most common. The mean size of masses was 4.8 cm. Pathologically, thick hyalinized collagen fibers surrounding large blood vessels and calcification were observed (81.6% and 23.7%, respectively). Multinucleated giant cells resembling Warthin–Finkeldey cell were also observed in occasional cases (23.7%). This is a unique paper that summarizes detailed clinical and pathological findings of a large series of a rare disease. The clinical information presented in this paper is more plausible than previous views and is useful for accurate diagnosis and understanding of the disease.

• Castleman disease
• abdominal cavity
• hyaline vascular type
• mediastinum
• unicentric Castleman disease

Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Considering the risks associated with a delayed diagnosis or misdiagnosis and the potential for overlap in clinical features and treatment, evaluation for suspected pemphigus disease often requires thorough clinical assessment and laboratory testing. Diagnosis is focused on individual biopsies for histopathology and direct immunofluorescence. Additional laboratory methods used for diagnosis include indirect immunofluorescence and enzyme-linked immunosorbent assay. Recent advancements, including anti-CD20 therapy, have improved the efficacy and reduced the morbidity of pemphigus treatment. This contribution presents updates on the pathophysiology, clinical features, diagnostic work-up, and medical management of pemphigus. Improved strategies for diagnosis and clinical assessment are reviewed, and newer treatment options are discussed.

• Autoimmune Diseases
• Enzyme-Linked Immunosorbent Assay
• Fluorescent Antibody Technique, Indirect
• Humans
• Pemphigus/diagnosis
• Pemphigus/drug therapy
• Skin Diseases, Vesiculobullous

• Castleman disease
• bilateral lung transplantation
• bronchiolitis obliterans
• paraneoplastic pemphigus

• PTLD
• antibodies
• children
• intestine transplant
• liver
• malignancy
• organ transplantation
• paraneoplastic pemphigus

• castleman's disease
• mucosal ulcers
• paraneoplastic pemphigus

• Adult
• Castleman Disease/diagnosis
• Castleman Disease/pathology
• Fatal Outcome
• Female
• Humans
• Paraneoplastic Syndromes/diagnosis
• Paraneoplastic Syndromes/pathology
• Pemphigus/diagnosis
• Pemphigus/pathology
• Pregnancy
• Pregnancy Complications, Neoplastic/diagnosis
• Pregnancy Complications, Neoplastic/pathology
• Uveitis/diagnosis
• Uveitis/pathology

• ACE, angiotensin-converting enzyme
• AIBD, autoimmune bullous disease
• CAAR, chimeric autoantibody receptor
• CI, confidence interval
• DSG, desmoglein
• EADV, European Academy of Dermatology and Venereology
• EC, extracellular
• EMA, European Medicines Agency
• FS, fogo selvage
• HR, hazard ratio
• ICD, International Classification of Diseases
• PF, pemphigus foliaceus
• PNP, paraneoplastic pemphigus
• PV, pemphigus vulgaris
• SMR, standardized mortality ratio

To explore the clinical features and immunological mechanisms of Castleman disease (CD) complicated with autoimmune diseases (AID).

We explored the prevalence and clinical manifestations of CD complicated with AID by reviewing clinical, pathological, and laboratory data of 40 CD patients retrospectively, and then explored abnormal immune mechanisms in the co-existence of the two entities by monitoring lymphocyte subsets in peripheral blood.

Paraneoplastic pemphigus, autoimmune hemolytic anemia, Sjogren’s syndrome, myasthenia gravis, and psoriasis were found to be coexisted with CD in 9/40 (22.5%) patients with different sequence of onset. No bias in the clinical and histological type of CD was observed for the occurrence of AID. CD patients with AID were more likely to have skin and/or mucous membrane damage and pulmonary complications, and presented elevated erythrocyte sedimentation rate, hypergammaglobulinemia, and positive autoantibodies than those without AID (p < 0.05). Deregulated cellular and innate immune responses as indicated by decreased CD3⁺ T cells and increased natural killer cells were observed in peripheral blood of CD patients with AID (p < 0.05). UCD patients with AID were successfully treated with surgery and immunosuppressive therapy. MCD complicated by AID relieved with immunosuppressors, cytotoxic chemotherapy, and rituximab.

Systemic inflammation/immunological abnormalities and organ dysfunction were associated with the occurrence of AID in CD. Impairment of cellular and innate immunity may be a candidate etiology for the coexistence of the two entities.

• Autoimmune diseases
• Castleman disease
• Immunological abnormalities
• Natural killer cells
• T cells

Paraneoplastic pemphigus (PNP) is a severe autoimmune syndrome commonly triggered by neoplasms. The prognosis of CLL-associated PNP is dismal due to its refractory course and secondary infection and no standard treatment was recommended. We retrospectively reported six CLL with PNP cases from 842 cases of CLL including diagnosis, treatment and prognosis. The median time between the initial of CLL to PNP was 36 months while the median overall survival from the diagnosis of PNP was 26 months. And three cases died of lung infection while 5 developed pulmonary symptoms. And 5 cases received fludarabine-based chemotherapy before developing PNP, which suggesting fludarabine was one of potential causes of PNP. For the treatment, five patients were rescued by combined regimens including rituximab, methylprednisolone, immunoglobulin, fresh frozen plasma and the last received ibrutinib combined with short-term prednisone. Fludarabine-based regimen may be one of the potential causes of PNP. The combined regimen might shed a new light, while ibrutinib is a promising drug for CLL with PNP, but needs much more evidence. PNP should be carefully treated to guide early diagnosis and intervention for a better prognosis.

Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease associated with an underlying malignancy. The survival rate at 5 years is reported to be as low as 38%. Thalidomide is a medication with strong anti‐inflammatory, immunomodulatory, antiangiogenic, and sedative properties. Recently, the successful application of thalidomide in several dermatological and hematological disorders inspired us to investigate its potential as an adjuvant treatment for PNP. Here, we report our experience of trial thalidomide therapy in 14 PNP patients. After treatment of their associated tumors, the patients were administered thalidomide (75‐100 mg/d) combined with or without low‐ to mid‐dose oral prednisone. Twelve patients completed the therapy. Seven patients (58%) achieved complete remission with no relapse, including two patients who received thalidomide monotherapy. Five patients (42%) died within 1 to 3 months. The 1‐year and 2‐year survival rates in our case series were 58% and 55%, respectively. The regimen was well tolerated. Although the treatment experience presented has a limited sample size and no control, our results imply that thalidomide may be an effective, safe, and economical treatment option for PNP patients. Further research is needed to better understand the mechanisms of action of thalidomide in PNP.

• Castleman disease
• paraneoplastic pemphigus
• thalidomide

• Castleman disease
• Hyaline vascular variant
• Paraneoplastic pemphigus
• Stroma-rich feature
• Survival analysis

• Giant lymph node hyperplasia
• paraneoplastic pemphigus (PNP)
• retroperitoneal
• surgical resection

• Bronchiolitis obliterans
• Castleman disease
• Thoracic

• Bronchiolitis Obliterans
• Castleman Disease
• Humans
• Lymph Nodes
• Prognosis
• Retrospective Studies

• Adrenal Cortex Hormones/adverse effects
• Azathioprine/therapeutic use
• Desmoglein 1
• Enzyme-Linked Immunosorbent Assay
• Humans
• Immunoglobulin G/immunology
• Immunosuppressive Agents/therapeutic use
• Pemphigus/classification
• Pemphigus/diagnosis
• Pemphigus/drug therapy
• Pemphigus/pathology
• Rituximab/therapeutic use
• Treatment Outcome

• Associations
• Comorbidities
• Epidemiology
• IgA pemphigus
• Mortality
• Paraneoplastic pemphigus
• Pemphigus
• Pemphigus foliaceus
• Pemphigus herpetiformis
• Pemphigus vulgaris

Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Circulating antibodies can be detected via indirect immunofluorescence (IIF) microscopy of different tissue substrates including human skin, monkey esophagus, and more recently, recombinant forms of the different target antigens. Latter are also employed in various commercial ELISA systems and by immunoblotting in in-house assays available in specialized laboratories. ELISA systems are also particularly valuable for monitoring of the disease activity during the disease course which can be helpful for treatment decisions. Exact diagnosis is essential for both treatment and prognosis, since some AIBD are associated with malign tumors such as paraneoplastic pemphigus and anti-laminin 332 mucous membrane pemphigoid. This review presents clinical and immunopathological features of AIBD for the state-of the art diagnosis of these disorders.

• ELISA
• autoantibody
• biochip
• dermatitis herpetiformis
• epidermolysis bullosa acquisita
• immunofluorescence
• pemphigoid
• pemphigus

• Paraneoplastic pemphigus
• Pemphigus paranéoplasique
• bullous disease
• maladie bulleuse
• paraneoplastic disease
• paranéoplasie

Bronchiolitis obliterans (BO) and paraneoplastic pemphigus are rare and ominous complications of Castleman disease. Collectively, these processes have been reported as part of paraneoplastic autoimmune multiorgan syndrome (PAMS), and they can occur in the setting of various hematologic malignant tumors, carcinoid tumors, and melanoma. Irrespective of the underlying malignancy driving PAMS, the clinical outcomes are uniformly poor, and there are no standard treatment regimens, given the clinical rarity of the syndrome. We describe 2 patients with unicentric Castleman disease complicated by paraneoplastic pemphigus and bronchiolitis obliterans. In addition to primary surgical resection for Castleman disease, we also used therapy from a treatment protocol used for bronchiolitis obliterans resulting from hematopoietic stem cell transplant (HSCT). We were able to treat the patients using intravenous immunoglobulin; rituximab; fluticasone, azithromycin, and montelukast (FAM); and rosuvastatin therapy. One patient demonstrated a favorable response, while the other demonstrated minimal response to this therapy.

• Bronchiolitis
• Chest imaging
• Leukotriene receptor antagonist
• Multisystemic syndrome
• Small airway disease

• Bronchiolitis obliterans
• Follicular lymphoma
• Paraneoplastic pemphigus
• R-CHOP

• Castleman disease
• ELISA, enzyme-linked immunosorbent assay
• IIF, indirect Immunofluorescence
• PNP, paraneoplastic pemphigus
• PV, pemphigus vulgaris
• autoimmune skin disease
• bullous disease
• direct and indirect immunofluorescence
• paraneoplastic pemphigus
• pemphigus vulgaris

Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems. PNP presents most frequently between 45 and 70 years old, but it also occurs in children and adolescents. A wide variety of lesions (florid oral mucosal lesions, a generalized polymorphous cutaneous eruption, and pulmonary involvement) may occur in patients with PNP. The earliest and most consistent finding is severe stomatitis. There is a spectrum of at least five clinical variants with different morphology. Similarly, the histological findings are very variable. Investigations to diagnose PNP should include checking for systemic complications (to identify tumor), skin biopsies (for histopathological and immunofluorescence studies), and serum immunological studies. PNP is characterized by the presence of autoantibodies against antigens such as desmoplakin I (250 kD), bullous pemphigoid aniygen I (230 kD), desmoplakin II (210 kD), envoplakin (210 kD), periplakin (190 kD), plectin (500 kD), and a 170 kD protein. Unlike other forms of pemphigus, PNP can affect other types of epithelia, such as gastrointestinal and respiratory tract. Treatment of PNP is difficult, and the best outcomes have been reported with benign neoplasms that have been surgically excised. The first-line treatment is high-dose corticosteroids with the addition of steroid-sparing agents. Treatment failures are often managed with rituximab with or without concomitant intravenous immunoglobulin. In general, the prognosis is poor, not only because of eventual progression of malignant tumors but also because treatment with aggressive immunosuppression therapy often results in infectious complications, which is unfortunately at this time the most common cause of death in PNP.

• direct and indirect immunofluorescence
• mucositis
• paraneoplastic pemphigus
• rituximab

Castleman's disease is a rare, benign lymphoproliferative disorder of unknown origin. Paraneoplastic pemphigus is a common association which presents as oral mucosal ulcerations. Abdominal and retroperitoneal Castleman's disease present either as a localized disease or as a systemic disease. We hereby present a 15-year-old male patient with oral mucosal lesions with localized vague right lower abdominal mass who was diagnosed to have Castleman's disease with paraneoplastic pemphigus which was surgically excised.

• Abdominal mass
• Castleman's disease
• paraneoplastic
• pemphigus