A case of relapsing polychondritis associated with auricular cartilage infiltration of immunoglobulin G4-positive plasma cells and lung cancer

Horai, Yoshiro; Miyamura, Tomoya; Arase, Hokuto; Kimura, Daisaku; Shimada, Kazuaki; Ishida, Motoko; Takahama, Soichiro; Kiyasu, Junichi; Minami, Ryoji; Yamamoto, Masahiro; Suematsu, Eiichi; Nakayama, Yoshihito

doi:10.1007/s10165-011-0439-2

Cited by 5 publications

(4 citation statements)

References 19 publications

(20 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Notably, there was a high number of IgG4 plasma cells present albeit below the number required for IgG4-RD diagnosis (Figure 2(b); 216 IgG4 + plasma cells, 601 IgG + plasma cells, IgG4/IgG ratio � 36%). Horai et al also presented a case of a 79-year-old man with RP with IgG4-expressing plasmacytes present in his left auricular cartilage [22]. ese cases of RP and concomitant IgG4-RD suggest that the mechanisms that promote development of IgG4-RD and RP may be shared.…”

Section: Discussionmentioning

confidence: 99%

Sudden Cardiac Death due to Coronary Artery Vasculitis in a Patient with Relapsing Polychondritis

Bukiri

Ruhoy

Buckner

2020

Case Reports in Rheumatology

View full text Add to dashboard Cite

Relapsing polychondritis (RP) is a systemic autoimmune disease characterized by relapsing and remitting inflammation of the cartilaginous structures of the ears, nose, tracheobronchial tree, and joints. Diagnosis is challenging due to the heterogeneity of clinical manifestations, the relapsing and remitting nature of the disease, the presence of coexistent diseases in at least one-third of patients, and the lack of a diagnostic blood test. Although RP-associated cardiac disease is the second most common cause of death behind tracheobronchial complications, coronary artery vasculitis is rare. This report describes a case of sudden cardiac death due to vasculitis affecting the coronary arteries in a patient with RP. The pathologic findings included obliterative coronary arteritis with plasma cells and storiform fibrosis, features suggesting that IgG4-related disease (IgG4-RD) may have contributed to the patient’s cardiac disease. The literature on vasculitis and cardiac disease in RP and the possible role of IgG4-RD in this setting is also reviewed. The primary take-home message from this case report is the importance of frequent screening for cardiac disease, regardless of symptoms, in patients with RP. In addition, considering the diagnosis of IgG4-RD in some cases thought to be RP may also be warranted.

show abstract

Section: Discussionmentioning

confidence: 99%

Sudden Cardiac Death due to Coronary Artery Vasculitis in a Patient with Relapsing Polychondritis

Bukiri

Ruhoy

Buckner

2020

Case Reports in Rheumatology

View full text Add to dashboard Cite

show abstract

“…The patient was found to have lung squamous cell carcinoma during hospitalization for RP. 11 It is believed that the pathogenesis of RP may not be lung cancer paraneoplastic syndrome because lung cancer surgery does not change the occurrence and development of RP, and no IgG4-positive cells are found in the pathology of specimens after lung cancer resection. It is unclear whether there is a direct relationship between the occurrence of RP and lung cancer.…”

Section: Discussionmentioning

confidence: 99%

Small cell lung cancer with relapsing polychondritis: A report of one case and the review of literature

Liu

Wang

et al. 2022

Int J Immunopathol Pharmacol

View full text Add to dashboard Cite

The present study reports the clinical data of a patient with small cell lung cancer who developed relapsing polychondritis. We report a case of a 57-year-old female presented with cough, expectoration, and fever. A Computed Tomography (CT) scan performed at the hospital revealed diffuse thickening of bronchial walls in both lungs. Bronchoscopy revealed that the tracheal mucosa was thickened, narrowed, and collapsed, and the bronchoscope could pass through. The bronchial mucosa on both sides was thickened and edematous, the surface was rough, each bronchus was narrow, and the intervertebral ridges were widened. Needle biopsy: considering small cell carcinoma in combination with immunohistochemical results. Her symptom was not improved after anti-infective therapy. The left auricle was red and swollen, the auricle collapsed, and the left eye had subconjunctival hemorrhage during her hospitalization without obvious cause. After multidisciplinary consultation, pulmonary small cell lung cancer cT0N2Mx rumen lymph node metastasis and RP were considered. Treatment: Prednisone, orally for RP. Chemotherapy combined with radiotherapy was given for small cell lung cancer. The chemotherapy regimen was carboplatin combined with etoposide. The patient has already been followed for 1 year after receiving chemoradiotherapy; the condition of the patient is stable at present. Based on the case of our patient, for cases of RP with symptoms such as auricle chondritis, ocular inflammatory disease, and nasal chondritis, we should pay great attention to whether the case is caused by lung cancer with relapsing polychondritis. Because of the rarity of the disease, the clinician should improve the recognition of the disease in order to strive for early diagnosis and therapy.

show abstract

“…In 2011, Horai et al 19 published a case report suggesting an alternative mechanism of RP progression focused primarily on IgG4 expressing plasmacytes. In a 79-year-old male patient diagnosed with RP, researchers found auricular cartilage destruction with high levels of infiltrative IgG4 expressing plasmacytes, accounting for 94.5% of the total IgG identified with immunohistochemical staining.…”

Section: Discussionmentioning

confidence: 99%

Relapsing Polychondritis as a Cause of Sudden and Unexpected Death With Central Nervous System Involvement

Lokken

Wang²

2022

Am J Forensic Med Pathol

View full text Add to dashboard Cite

Relapsing polychondritis (RP) is a rare inflammatory disease process that affects cartilaginous tissues throughout the body. Although the pathogenesis remains unknown, RP is thought to be an autoimmune disorder in which host immune cells are conditioned to attack the body's cartilage, such as the ears, nose, eyes, joints, and airways, resulting in inflammation and destruction of otherwise healthy tissues. In rare and unusual cases, neurological involvement has been described.We report a case of a 36-year-old man with a medical history of asthma and suspected seronegative rheumatoid arthritis/RP and panuveitis who was found deceased in his residence. Postmortem examination revealed cartilaginous destruction of the external ear and large airways and meningoencephalitis involving the left medial temporal lobe without an underlying infectious cause.Progressive destruction of airway tissue and increased susceptibility to pulmonary infection is the most common cause of death in RP. Central nervous system involvement is exceedingly rare, presenting with highly variable clinical and pathological manifestations. A review of RP and systemic manifestations will follow. Accurate recognition of this multisystem autoimmune disease as a cause of sudden and unexpected death is critical for proper death certification and to broaden our understanding of this disease.

show abstract

A case of relapsing polychondritis associated with auricular cartilage infiltration of immunoglobulin G4-positive plasma cells and lung cancer

Cited by 5 publications

References 19 publications

Sudden Cardiac Death due to Coronary Artery Vasculitis in a Patient with Relapsing Polychondritis

Sudden Cardiac Death due to Coronary Artery Vasculitis in a Patient with Relapsing Polychondritis

Small cell lung cancer with relapsing polychondritis: A report of one case and the review of literature

Relapsing Polychondritis as a Cause of Sudden and Unexpected Death With Central Nervous System Involvement

Contact Info

Product

Resources

About