시장보고서
상품코드
1705100

IgG4 관련 질환 시장 - 시장 인사이트, 역학, 시장 예측(2034년)

IgG4-Related Disease - Market Insight, Epidemiology, and Market Forecast - 2034

발행일: | 리서치사: DelveInsight | 페이지 정보: 영문 200 Pages | 배송안내 : 2-10일 (영업일 기준)

    
    
    




■ 보고서에 따라 최신 정보로 업데이트하여 보내드립니다. 배송일정은 문의해 주시기 바랍니다.

주요 하이라이트

  • IgG4-RD는 췌장, 담관, 침샘, 눈물샘, 안와, 신장, 폐, 후복막 등 거의 모든 장기에 발병할 수 있습니다.
  • IgG4-RD의 실제 유병률은 알려지지 않았으며, IgG4-RD의 발견이 비교적 최근에 발견되었고, 널리 인식되지 않았으며, 종종 서서히 발현되기 때문에 인지도가 낮고 보고율이 낮은 것으로 추정됩니다. 남성이 우세하며, 대규모 코호트에서 진단 당시 평균 연령은 30대에서 50대에 이르는 것으로 나타났습니다. 그러나 소아 환자에서도 고전적인 사례가 관찰되고 있습니다.
  • 2024년 주요 7개국에서 진단된 IgG4-RD 유병자 수는 약 14만 8,970명입니다. 이 중 미국이 IgG4-RD 진단을 받은 유병자 수가 가장 많습니다.
  • 미국, EU 4개국, 영국의 시나리오와 달리 일본에서는 진단 유병률을 보여주는 여러 연구가 진행되고 있습니다. 따라서 2015년부터 2018년까지 IgG4 관련 질환의 진단이 급격히 증가했다는 증거와 함께 일본 국가 데이터베이스의 10년간 누적 진단 유병률 추정치에 따르면, 2024년 일본 내 진단 유병률은 약 38,690건에 달할 것으로 추정됩니다.
  • IgG4로 진단받은 환자의 약 20-30%는 전신요법별 치료를 받지 않고 수술을 선택하거나 경과를 관찰하고 있습니다.
  • IgG4-RD는 병태생리가 불분명하기 때문에 특이적인 치료제는 없으며, 승인된 치료법도 없습니다. 주요 치료는 프레드니손과 같은 부신피질스테로이드 약물에 의한 염증 및 증상 관리입니다. 재발하거나 효과가 없는 경우, 스테로이드 의존성을 줄이기 위해 아자티오프린이나 메토트렉세이트와 같은 면역억제제를 사용하기도 합니다. 항 CD20 단클론항체인 리툭시맙도 염증을 억제하고 병의 진행을 늦추는 효과가 있습니다. 글루코코르티코이드는 여전히 1차 선택 약물입니다.
  • 현재 제한적인 파이프라인에는 아플리주나(Amgen), 오벡셀리맙(Zenas BioPharma, Bristol Myers Squibb), 릴자브루티닙(Sanofi) 등 3개의 새로운 치료제가 있습니다. 이 중 애플리주나와 오벡셀리맙은 서로 경쟁할 것으로 예상됩니다.
  • 2025년 4월에 승인될 것으로 예상되는 업질나는 IgG4-RD 시장을 변화시킬 것으로 예상되며, 이 미충족 치료 질환에 대해 승인된 최초의 치료제가 될 것입니다. 그것은 치료의 전망을 재정의하고, 더 높은 진단율을 촉진하고, 가격 책정 및 의사 채택을 통해 상업적 역학을 형성할 것으로 보입니다. 그러나 시장 교육, 실제 임상 검증, 미래의 경쟁은 장기적인 영향에 영향을 미칠 것으로 보입니다.
  • 릴자브루티닙은 B세포 신호전달 경로를 표적으로 하는 유망한 경구용 2상 치료제입니다. 만약 승인된다면, 보다 간편한 자가 투여 옵션을 제공함으로써 정맥주사/SC 생물학적 제제 시장에 지각변동을 일으킬 수 있습니다.
  • 2024년 주요 7개국에서 IgG4-RD의 총 시장 규모는 약 1억 7,000만 달러에 달했습니다. 이 시장 규모는 파이프라인에 있는 잠재적인 신약이 승인되면 크게 성장할 것으로 예상됩니다.

본 보고서는 IgG4 관련 질환 시장을 조사하여 미국, EU 4개국(독일, 프랑스, 이탈리아, 스페인, 영국, 일본의 시장 동향과 함께 과거 역학 및 예측에 대한 상세한 이해를 제공합니다. 현재 치료법, 신약, 개별 치료법의 시장 점유율, 2020년에서 2034년까지 주요 7개국의 현재 및 예측 시장 규모를 제공합니다. 또한, 현재 치료법/알고리즘, 미충족 의료 수요를 포괄하여 최적의 기회를 발굴하고 시장 잠재력을 평가합니다.

IgG4-RD는 혈장 내 IgG4 농도의 상승 유무에 관계없이, 감염된 조직에서 IgG4 양성 형질세포의 밀집 침윤을 특징으로 하는 전신성 섬유성 염증성 질환입니다. 만성 섬유성 염증성 질환으로 다양한 장기에 영향을 미칩니다. 혈청 IgG4 농도의 상승과 IgG4 발현 형질세포의 풍부한 침윤은 이 자가면역질환의 주요 진단적 특징이며, IgG4-RD의 흔한 장기 병변은 침샘, 췌장 및 담관이며, 간 병변은 잘 알려져 있지 않습니다.

IgG4-RD는 미쿨리츠병, 자가면역성 췌장염, 갑상선기능저하증, 리델 갑상선염, 간질성 폐렴, 간질성 신염, 전립선염, 림프절 부종, 후복막 섬유증, 염증성 대동맥류, 염증성 가성종양 등 다양한 질환을 앓고 있는 환자에서 일정 비율로 발견될 수 있습니다.

IgG4-RD는 여러 장기를 동시에 침범할 수 있기 때문에 진단이 어렵습니다. 진단을 위해서는 임상 검사, 내시경 검사, X선 검사, 혈청학적 검사, 장기 병변 및 말초 장기 장애(호르몬 균형 장애 등)를 검사해야 합니다. 조직 진단을 위해서는 피부를 포함한 환부 조직의 생검이 필요합니다. 혈액 검사에서 혈청 IgG4 농도의 상승과 말초 호산구 증가가 관찰될 수 있습니다.

혈청 IgG4 농도는 가장 중요한 바이오마커로, 최대 90%의 환자에서 높은 수치를 보이지만, 그 수치는 다양하며, IgG4 농도의 감소는 종종 치료 효과를 나타내지만, 장기간 생존하는 형질세포로 인해 관해기에도 정상으로 돌아오지 않을 수 있습니다. IgG1, IgE, 호산구 증가, 보체 수준(C3 및 C4)과 같은 다른 바이오마커도 질병 활동을 반영할 수 있으며, IgG4 관련 신장 질환에서 저보체혈증은 두드러지게 나타납니다. 보혈증은 두드러지게 나타납니다.

IgG4-RD의 경우 치료가 반드시 필요한 것은 아니며, 무증상인 경우 주의 깊게 지켜보는 것이 적절한 경우도 있습니다. 반면, IgG4-RD는 주요 장기의 기능부전이나 장애를 유발할 수 있기 때문에 중요한 장기가 관련되어 있거나 환자가 증상을 보이는 경우 신속한 치료가 필요합니다.

IgG4-RD 치료에 대한 국제적인 합의문에 따르면, 글루코코르티코이드는 대부분의 췌장 및 췌장 외 질환에서 극적인 임상적 효과가 있기 때문에 일차 선택 약물입니다. 따라서 다양한 글루코코르티코이드를 보존하는 약물이 다양한 해부학적 영역에서 관해 유지 약물로 사용되고 있으며(예: 아자티오프린, 모페틸 미코페놀산, 메토트렉세이트, 사이클로포스파미드, 보르테조밉), 그 결과는 다양합니다.

아자티오프린, 모페틸 미코페놀산, 메토트렉세이트와 같은 스테로이드 보존 약물의 사용은 글루코코르티코이드의 부작용을 줄이고 관해 상태를 지속시키기 위해 사용되지만, 그 효과는 충분히 입증되지 않았습니다. 리툭시맙 요법은 CD20 양성 형질세포 전구체를 고갈시키는 최근의 유망한 대체 요법입니다. 형질 모세포가 감소하면 IgG4 합성이 감소합니다.

글루코코르티코이드 치료에 대한 빠른 반응이 특징적인 소견으로 지적된 AIP의 초기 기술 이후, 글루코코르티코이드는 IgG4-RD의 일차 치료제로 간주되어 왔습니다. 발표된 IgG4-RD 관리에 관한 합의문에 따르면, 대다수의 IgG4-RD 전문가들은 치료되지 않은 활동성 질환에 대한 1차 선택 약물이 글루코코르티코이드라고 믿고 있습니다. 프레드니손은 관해 도입의 첫 단계로 자주 사용됩니다. 대부분의 환자는 글루코코르티코이드 치료에 며칠에서 몇 주 이내에 반응하며, 대부분은 몇 달 이내에 관해에 도달합니다.

  • 2024년 미국 내 IgG4-RD 유병자 수는 약 5만 6,800명이며, 예측 기간(2025-2034년) 동안 증가할 것으로 예상됩니다.
  • 2024년 미국 내 성별 진단 유병자 수는 남성 약 3만 9,100명, 여성 약 17,700명입니다.
  • 2024년 EU 4개국과 영국에서 IgG4-RD 진단을 받은 유병자 수는 약 5만 3,550명으로 집계됐습니다. 남성은 약 3만 8,850명, 여성은 1만 4,750명이었습니다.
  • 연령대 분류는 0-17세, 18-29세, 30-44세, 45-64세, 65-74세, 75세 이상 등 6가지 연령대를 고려했습니다. 분석 결과, 일본에서는 75세 이상 연령층에서 높은 유병자 비율이 관찰되었으며, 2024년 일본의 연령별 IgG4-RD 유병자 수는 75세 이상에서 약 1만 2,800명이 진단을 받은 것으로 나타났습니다. 예측 기간(2025-2034년) 동안 환자 수는 증가할 것으로 예상됩니다.

오벡셀리맙(XmAb5871) : 제나스 바이오파마와 브리스톨 마이어스 스퀴브(Bristol Myers Squibb)

오벡세리맙(XmAb5871)은 B세포계에 널리 존재하는 CD19와 FcRIIb에 모두 결합하도록 설계된 이중 기능성 단클론항체로, 많은 자가면역질환에 관여하는 세포의 활성을 고갈시키지 않고 억제합니다. 이 독특한 작용 기전과 자가 투여 가능한 SC 주사 요법은 만성 자가면역질환에서 B세포 계열의 병원성 역할에 광범위하고 효과적으로 대처할 수 있는 잠재력을 가지고 있습니다.

현재 Zenas는 IgG4-RD, 다발성 경화증, 전신성 홍반성 루푸스 및 온열성 자가면역성 용혈성 빈혈을 포함한 여러 자가면역질환에서 오벡세리맙에 대한 여러 임상 II상 및 III상 시험을 진행하고 있습니다. 류마톨로지(Rheumatology)지는 IgG4-RD 환자를 대상으로 오벡세리맙의 치료 효과를 평가한 임상 2상 결과를 발표했습니다. 이러한 결과를 바탕으로 IgG4-RD 환자를 대상으로 오벡세리맙을 SC 주사제로 투여했을 때 효과와 안전성을 추가적으로 검토하기 위한 임상 3상 시험이 진행 중입니다.

아플리자나(성분명: 이네비리주맙) : Amgen

업리자나는 인간화 단클론항체로서, 기저질환 과정에 기여하는 주요 세포(형질모세포 및 일부 형질세포를 포함한 자가항체를 생산하는 CD19 B세포)를 표적으로 하여 지속적으로 고갈시키는 역할을 합니다. 환자는 두 번의 초기 주입 후 6개월마다 한 번씩 애플리자나를 투여해야 합니다. 애플리주나는 현재 시신경척수염 치료제로 승인됐습니다. 현재 이 약은 임상 3상 시험을 완료했으며, FDA는 PDUFA 발효일을 2025년 4월 3일로 정했습니다.

애플리주나는 현재 IgG4-RD에 대한 치료 옵션의 심각한 격차를 해소하고, 환자에게 큰 혜택을 제공하는 동시에 이 복잡하고 종종 쇠약해지는 증상을 관리하기 위해 승인된 최초의 핵심 치료제가 될 것으로 기대됩니다. 향후 오벡세리맙이 승인되어 이중기능 표적 치료제로 경쟁할 가능성이 있습니다.

현재 IgG4-RD의 신약 현황은 세 가지 약물이 있습니다. 그 중 CD-19를 표적으로 하는 것은 오벡세리맙(이기능성)과 애플리주나로, B세포에 존재하는 단백질인 CD19를 표적으로 하는 치료는 IgG4-RD의 발병에 관여하는 것으로 여겨지는 B세포를 감소시킴으로써 IgG4-RD의 치료제로서 유망한 치료법입니다. 특히, CD19를 표적으로 하는 항체인 애플리자나는 IgG4-RD 환자의 재발을 억제하고 관해에 도달하는 효과를 입증했습니다.

IgG4-RD의 병인은 아직 밝혀지지 않았으며, 현재 연구 중이기 때문에 특정 약물로 치료할 수 없으며, 현재 IgG4-RD 환자를 위해 승인된 치료제는 시판되고 있지 않습니다. 최근 발표된 "IgG4-RD 관리 및 치료에 관한 국제 합의 지침 및 가이드라인"은 IgG4 수치로 인한 질병을 식별하는 기준과 환자 치료에 대한 권장 사항을 상세히 설명합니다.

활동성 또는 치료받지 않은 IgG4-RD 환자는 일반적으로 글루코코르티코이드를 1차 선택 약제로 투여합니다. 병의 중증도와 긴급성에 따라 다르지만, 관해 유도에는 프레드니손 30-40mg/일 또는 체중 조절 용량으로 0.6mg/kg을 매일 투여하는 경우가 많습니다.

경우에 따라 IgG4-RD의 치료에는 기존의 "질병 조절 항류마티스제제"(DMARDs)가 사용됩니다. 형질세포는 모든 류마티스 질환에서 효과적인 바이오마커가 될 수 있는 최초의 세포이며, B세포의 맞춤형 치료는 질병 활동을 추적하기 위해 총 형질세포 농도를 이용합니다.

IgG4-RD를 관리하기 위한 수많은 잠재적 치료법이 조사되고 있기 때문에 예측 기간 동안 치료 영역이 크게 재편될 것임에 틀림없습니다. 그러나 가격 책정 및 상환과 관련된 문제가 이 모든 파이프라인 치료제의 운명과 전체 수익에 미치는 영향을 결정하게 될 것입니다.

Zenas BioPharma, Amgen, Sanofi와 같은 주요 개발사들은 각기 다른 임상 개발 단계에서 주요 후보물질을 평가하고 있습니다. 이들 회사는 IgG4-RD를 치료하기 위해 자사 제품을 연구하는 것을 목표로 하고 있습니다.

본 보고서는 주요 7개국의 IgG4 관련 질환 시장을 조사하여 시장 개요와 함께 역학, 환자 동향, 새로운 치료법, 2034년까지의 시장 규모 예측, 미충족 의료 수요 등을 조사하여 전해드립니다.

목차

제1장 중요한 인사이트

제2장 보고서 소개

제3장 주요 요약

제4장 주요 사건

제5장 역학과 시장 예측 조사 방법

제6장 IgG4-RD시장 개요

  • IgG4-RD 치료제 시장 점유율(%) 분포, 2024년
  • IgG4-RD 치료제 시장 점유율(%) 분포, 2034년

제7장 질환 배경과 개요

  • 소개
  • 임상 표현형
  • IgG4-RD 관련 조직학
  • IgG4-RD 관련 병태
  • 질환 아류형과 임상 증상
  • IgG4-RD 관련 징조와 증상
  • IgG4-RD 진단 평가
  • IgG4-RD 치료와 관리
  • IgG4-RD 가이드라인, 추천사항 및 관리

제8장 주요 7개국의 IgG4-RD 역학과 환자 인구

  • 주요 조사 결과
  • 가정과 근거
  • 주요 7개국의 IgG4-RD 진단된 유병 증례 총수
  • 미국
  • EU 4개국과 영국
  • 일본

제9장 환자 동향

제10장 신흥 약제

  • 키 크로스 경쟁
  • Obexelimab(XmAb5871) : Zenas BioPharma와 Bristol Myers Squibb
  • UPLIZNA(inebilizumab) : Amgen
  • Rilzabrutinib(PRN1008/SAR444671) : Sanofi

제11장 IgG4-RD : 주요 7개국 시장 분석

  • 주요 조사 결과
  • 시장 전망
  • 주요 시장 예측 가정
  • 컨조인트 분석
  • 주요 7개국의 IgG4-RD 전체 시장 규모
  • 주요 7개국의 IgG4-RD 시장 규모(치료법별)
  • 미국
  • EU 4개국과 영국
  • 일본

제12장 미충족 수요

제13장 SWOT 분석

제14장 KOL의 견해

제15장 시장 접근과 상환

  • 미국
  • EU 4개국과 영국
  • 일본
  • IgG4-RD 상환

제16장 부록

제17장 DelveInsight의 서비스 내용

제18장 면책사항

제19장 DelveInsight 소개

ksm 25.04.22

Key Highlights:

  • IgG4-RD can affect virtually any organ, with common presentations including the pancreas, bile ducts, salivary glands, lacrimal glands, orbits, kidneys, lungs, and retroperitoneum.
  • The true prevalence of IgG4-RD is unknown. Given its relatively recent discovery, lack of widespread recognition, and frequently indolent presentation, it is likely that it is underrecognized and underreported. There is a male predominance, and the average age at diagnosis in large cohorts is in the thirties to fifties. However, classic cases have been observed in pediatric patients as well.
  • There were nearly 148,970 diagnosed prevalent cases of IgG4-RD in 7MM in 2024. Among these, the US accounted for the highest number of diagnosed prevalent cases of IgG4-RD.
  • Contrary to the scenario in the US and EU4, and the UK, there have been multiple studies conducted in Japan stating the diagnosed prevalence; therefore, based on 10-year cumulative diagnosed prevalence estimates from the National Database in Japan, along with evidence around drastic increase in the diagnosis of IgG4 related disease from 2015-2018, there were ~38,690 diagnosed cases in 2024 in Japan.
  • Almost 20-30% of the diagnosed IgG4 patients do not undergo any treatment by systemic therapies, as they opt for either surgery or are under a watchful waiting strategy.
  • There are no specific drugs to cure IgG4-RD due to unclear pathophysiology completely, and no approved therapies are available. The main treatment is corticosteroids like prednisone to manage inflammation and symptoms. If the disease relapses or doesn't respond, immunosuppressants like azathioprine or methotrexate may be used to reduce steroid dependence. Rituximab, an anti-CD20 monoclonal antibody, has also shown effectiveness in controlling inflammation and slowing disease progression. Glucocorticoids remain the first-line therapy.
  • The current constrained pipeline features three emerging therapies: UPLIZNA (Amgen), obexelimab (Zenas BioPharma and Bristol Myers Squibb), and rilzabrutinib (Sanofi). Among these emerging therapies, UPLIZNA and obexelimab are expected to compete with each other strongly.
  • The anticipated April 2025 approval of UPZILNA will transform the IgG4-RD market, marking the first-ever approved therapy for this underserved condition. It will redefine the treatment landscape, drive higher diagnosis rates, and shape commercial dynamics through pricing and physician adoption. However, market education, real-world validation, and future competition will influence its long-term impact.
  • Rilzabrutinib, a Phase II therapy, represents a promising oral therapy targeting B-cell signaling pathways. If approved, it could disrupt the IV/SC biologic market by offering a more convenient, self-administered alternative.
  • In 2024, the total market size of IgG4-RD in 7MM was nearly USD 170 million. The market size is expected to observe significant growth after the approval of potential emerging therapies in the pipeline.

DelveInsight's "IgG4-Related Disease (IgG4-RD) - Market Insights, Epidemiology and Market Forecast - 2034" report delivers an in-depth understanding of historical and forecasted epidemiology as well as market trends in the United States, EU4 (Germany, France, Italy, Spain) and the United Kingdom, and Japan. IgG4-RD market report provides current treatment practices, emerging drugs, market share of individual therapies, and current and forecasted 7MM market size from 2020 to 2034. The report also covers current treatment practices/algorithms and unmet medical needs to curate the best opportunities and assess the market's potential.

Geography Covered:

  • The United States
  • EU4 (Germany, France, Italy, and Spain) and the United Kingdom
  • Japan

Study Period: 2020-2034

IgG4-RD Understanding and Treatment

IgG4-RD Overview

IgG4-RD is a systemic fibro inflammatory disease characterized by dense infiltration of IgG4-positive plasma cells in the affected tissue(s) with or without elevated plasma levels of IgG4. It is a chronic fibroinflammatory disorder affecting a wide range of organs. Elevation of serum IgG4 concentrations and abundant infiltration of IgG4-expressing plasma cells are key diagnostic features of this autoimmune disease. Although common organ involvement of IgG4-RD includes the salivary glands, pancreas, and bile duct, hepatic involvement is less well established.

IgG4-RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz's disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor.

IgG4-RD Diagnosis

IgG4-RD is challenging to diagnose since it may simultaneously affect several organs. Clinical, endoscopic, radiographic, and serological testing are required to diagnose the illness to examine organ involvement and end-organ damage (such as hormonal imbalances). A biopsy of the afflicted organ tissues, including the skin, is necessary for tissue diagnosis. Blood testing may reveal elevated serum IgG4 levels and peripheral eosinophilia.

Serum IgG4 concentration is the most important biomarker, with elevated levels found in up to 90% of patients, though this can vary. While a drop in IgG4 levels often signals treatment response, it may never return to normal, even in remission, due to long-lived plasma cells. Elevated IgG4 can also occur in other conditions, so its value must be considered within the broader clinical context. Other biomarkers, such as IgG1, IgE, eosinophilia, and complement levels (C3 and C4), can also reflect disease activity, with hypocomplementemia notably seen in IgG4-related kidney disease.

IgG4-RD Treatment

In individuals with IgG4-RD, treatment is not always essential, and in some asymptomatic instances, attentive waiting is appropriate. IgG4-RD, on the other hand, can induce major organ malfunction and failure, necessitating prompt therapy when essential organs are implicated or individuals exhibit symptoms.

According to the International Consensus Statement on the treatment of IgG4-RD, glucocorticoids represent the first-line therapy because they lead to dramatic clinical responses in most cases with pancreatic and extra-pancreatic diseases. Thus, a variety of glucocorticoid-sparing agents have been employed in different anatomical districts as remission-maintenance drugs (e.g., azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, and bortezomib), with alternate results.

The use of steroid-sparing medications such as azathioprine, mycophenolate mofetil, and methotrexate are used to alleviate the adverse effects of glucocorticoids and to sustain remission; however, there is insufficient proof of their efficacy. Rituximab therapy is a more recent, promising alternative; it depletes CD20-positive plasmablast precursors; as there are fewer plasmablasts, IgG4 synthesis declines.

Since the earliest description of AIP, when a quick response to glucocorticoid treatment was noted as a distinctive finding, glucocorticoids have been regarded as the first-line therapy in IgG4-RD. The majority of IgG4-RD experts continue to believe that glucocorticoid is the first-line therapy for an active, untreated illness, according to the published consensus statement on the management of IgG4-RD. Prednisone is frequently used as the first step in remission induction. Most patients have a response to glucocorticoid treatment within a few days or weeks, and the majority of them reach remission within a few months.

IgG4-RD Epidemiology

As the market is derived using a patient-based model, the IgG4-RD epidemiology chapter in the report provides historical as well as forecasted epidemiology segmented by total diagnosed prevalent cases of IgG4-RD, gender-specific diagnosed prevalent cases of IgG4-RD, and age-specific diagnosed prevalent cases of IgG4-RD in the 7MM covering the United States, EU4 countries (Germany, France, Italy, Spain) and the United Kingdom, and Japan from 2020 to 2034.

  • In 2024, the total diagnosed prevalent cases of IgG4-RD in the United States were approximately 56,800 cases, projected to increase during the forecast period (2025-2034).
  • In 2024, gender-specific diagnosed prevalent cases accounted for approximately 39,100 and 17,700 cases for males and females, respectively, in the US.
  • The total diagnosed prevalent cases of IgG4-RD in EU4 and the UK were approximately 53,550 in 2024. Males accounted for approximately 38,850 cases, and 14,750 cases in females.
  • We have considered six age groups for the categorization of age groups, i.e., 0-17 years, 18-29 years, 30-44 years, 45-64 years, 65-74 years, and =75 years. As per our analysis, a higher percentage of diagnosed prevalent cases was observed in the age group =75 years in Japan. In 2024, the age-specific diagnosed prevalent cases of IgG4-RD in Japan were approximately 12,800 cases in the age group =75 years. The cases will increase during the forecast period (2025-2034).

Drug Chapters

The drug chapter segment of the IgG4-RD report encloses a detailed analysis of IgG4-RD marketed drugs and late-stage (Phase III and Phase II) pipeline drugs. It also helps understand the IgG4-RD clinical trial details, expressive pharmacological action, agreements and collaborations, approval and patent details, advantages and disadvantages of each included drug and the latest news and press releases.

Emerging Drugs

Obexelimab (XmAb5871): Zenas BioPharma and Bristol Myers Squibb

Obexelimab (XmAb5871) is a bifunctional monoclonal antibody designed to bind both CD19 and Fc?RIIb, which are broadly present across B-cell lineage, to inhibit the activity of cells that are implicated in many autoimmune diseases without depleting them. This unique mechanism of action and self-administered SC injection regimen may broadly and effectively address the pathogenic role of B-cell lineage in chronic autoimmune disease.

Currently, Zenas is conducting multiple Phase II and Phase III trials of obexelimab in several autoimmune diseases, including IgG4-RD, multiple sclerosis, systemic lupus erythematosus, and warm autoimmune hemolytic anemia. In August 2023, The Lancet Rheumatology published findings from a Phase II study evaluating obexelimab for the treatment of patients with IgG4-RD. Based on these results, a Phase III study in patients with IgG4-RD is ongoing to investigate further the efficacy and safety of obexelimab administered as an SC injection.

UPLIZNA (inebilizumab): Amgen

UPLIZNA is a humanized monoclonal antibody that causes targeted and sustained depletion of key cells that contribute to the underlying disease process (autoantibody-producing CD19+ B cells, including plasmablasts and some plasma cells). After two initial infusions, patients need one dose of UPLIZNA every 6 months. UPLIZNA is currently approved for the treatment of Neuromyelitis Optica. Currently, the drug has completed its Phase III results, and the FDA has granted a PDUFA action date of April 3, 2025.

UPLIZNA is anticipated to be the first approved cornerstone therapy for managing this complex and often debilitating condition, providing significant benefits to patients while addressing a critical gap in current treatment options for IgG4-RD. In the future, the drug could face competition with the approval of obexelimab, which operates through a bifunctional targeting mechanism of action.

Drug Class Insights

The current IgG4-RD emerging landscape has only three drugs. Among them, obexelimab (bifunctionally) and UPLIZNA target CD-19. Targeting CD19, a protein found in B cells, shows promise as a treatment for IgG4-RD by depleting B cells, which are thought to play a role in the disease's pathogenesis. Specifically, UPLIZNA, a CD19-targeted antibody, has demonstrated efficacy in reducing flares and achieving remission in IgG4-RD patients.

Market Outlook

As the etiology of IgG4-RD is unclear and still being studied, no particular medications can cure the disease. For people with IgG4-RD, there are no approved treatments on the market at this time. The criteria for identifying the disease caused by IgG4 levels and patient treatment recommendations were recently detailed in the guidelines released by the "International Consensus Guidance Statement on the Management and Treatment of IgG4-RD."

Patients with IgG4-RD that are active or untreated typically get glucocorticoids as their first line of therapy. Depending on the severity of the condition and the urgency of the situation, remission induction is frequently started with 30-40 mg/day of prednisone or a weight-adjusted dosage of 0.6 mg/kg of body weight each day.

In certain instances, conventional "Disease-Modifying Anti-Rheumatic Drugs" (DMARDs) are utilized to treat IgG4-RD. Plasmablasts are the first cell in any rheumatologic disorder to serve as an effective biomarker, and tailored therapy in B-cell employs total plasmablast concentration to track disease activity.

As numerous potential therapies are being investigated to manage IgG4-RD, it is safe to predict that the treatment space will experience significant reconstitution during the forecast period. However, the challenges of pricing and reimbursement accompanied by will decide the fate of all these pipeline therapies and the impact they will have on overall revenue generation.

Key players such as Zenas Biopharma, Amgen, and Sanofi are evaluating their lead candidates in different stages of clinical development, respectively. They aim to investigate their products to treat IgG4-RD.

  • In 2024, the US captured the highest market share, i.e., nearly USD 90 million out of all the 7MM countries.
  • EU4 and the UK accounted for nearly USD 80 million of the market share, and Germany accounted for the highest share in Europe in 2024.
  • In Japan, the IgG4-RD market size accounted for nearly USD 6 million and is expected to increase at a significant CAGR during the forecast period (2025-2034).
  • The lack of an established regulatory precedent and standardized endpoints for IgG4-RD trials poses approval risks. Additionally, payer resistance to high-cost biologics may slow adoption, especially if off-label rituximab remains a cost-effective alternative. However this will not be an issue in Japan as rituximab is not yet permitted for use to treat IgG4-RD in Japan due to Japanese medical insurance reasons.

Key Updates

  • In November 2024, Zenas BioPharma announced the completion of its targeted enrollment for the Phase III INDIGO trial of its lead product candidate, obexelimab, for the treatment of patients with IgG4-RD.
  • In November 2024, Zenas BioPharma announced that the company anticipates reporting topline results for the INDIGO trial by the end of 2025.
  • In August 2024, Amgen announced that the US FDA had granted Breakthrough Therapy Designation (BTD) to UPLIZNA for the treatment of IgG4-RD based on data from the MITIGATE study.
  • In February 2025, Amgen announced that the FDA had accepted the regulatory submission for the Phase III MITIGATE study under priority review, with a PDUFA action date of April 3, 2025.

IgG4-RD Drugs Uptake

This section focuses on the uptake rate of potential drugs expected to be launched in the market during 2020-2034. The landscape of IgG4-RD treatment has experienced a profound transformation with the uptake of novel drugs. These innovative therapies are redefining standards of care. Furthermore, the increased uptake of these transformative drugs is a testament to the unwavering dedication of physicians, oncology professionals, and the entire healthcare community in their tireless pursuit of advancing cancer care. This momentous shift in treatment paradigms is a testament to the power of research, collaboration, and human resilience.

IgG4-RD Pipeline Development Activities

The report provides insights into different therapeutic candidates in Phase III, Phase II/III, Phase II, Phase I/II, and Phase I. It also analyzes key players involved in developing targeted therapeutics.

Pipeline Development Activities

The report covers detailed information on collaborations, acquisitions and mergers, licensing, and patent details for IgG4-RD emerging therapies.

KOL Views

To keep up with current market trends, we take KOLs and SMEs' opinions working in the domain through primary research to fill the data gaps and validate our secondary research. Some of the leaders like MD, PhD, Research Project Manager, Director, and others. Their opinion helps to understand and validate current and emerging therapies and treatment patterns or IgG4-RD market trends. This will support the clients in potential upcoming novel treatments by identifying the overall scenario of the market and the unmet needs.

Delveinsight's analysts connected with 20+ KOLs to gather insights; however, interviews were conducted with 10+ KOLs in the 7MM. Centers such as the Massachusetts General Hospital, Hopital La Timone, Harvard Medical School, Hospital Universitari Vall d'Hebron, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, etc., were contacted. Their opinion helps understand and validate IgG4-RD epidemiology and market trends.

Qualitative Analysis

We perform qualitative and market Intelligence analysis using various approaches, such as SWOT and conjoint analysis. In the SWOT analysis, strengths, weaknesses, opportunities, and threats in terms of disease diagnosis, patient awareness, patient burden, competitive landscape, cost-effectiveness, and geographical accessibility of therapies are provided. These pointers are based on the analyst's discretion and assessment of the patient burden, cost analysis, and existing and evolving treatment landscape.

The analyst analyzes multiple emerging therapies based on relevant attributes such as safety, efficacy, frequency of administration, route of administration, and order of entry.

In efficacy, the trial's primary and secondary outcome measures are evaluated.

Further, the therapies' safety is evaluated wherein the acceptability, tolerability, and adverse events are majorly observed, and it sets a clear understanding of the side effects posed by the drug in the trials.

Market Access and Reimbursement

The cost of newly approved medications is usually high, and because of it, patients escape from proper treatment or opt for off-label and cheap medications. It affects market access to newly launched medications, and reimbursement is crucial. The decision to reimburse often comes down to the drug's price relative to the benefit it produces in treated patients. Market access and reimbursement options can differ depending on regulatory status, target population size, the setting of care, unmet needs, the magnitude of incremental benefit claims, and costs.

The report further provides detailed insights on the country-wise accessibility and reimbursement scenarios, cost-effectiveness scenario of approved therapies, programs making accessibility easier and out-of-pocket costs more affordable, insights on patients insured under federal or state government prescription drug programs, etc.

Scope of the Report:

  • The report covers a descriptive overview of IgG4-RD, explaining its causes, signs and symptoms, pathogenesis, and currently available therapies.
  • Comprehensive insight has been provided into IgG4-RD epidemiology and treatment.
  • Additionally, an all-inclusive account of both the current and emerging therapies for IgG4-RD is provided, along with the assessment of new therapies which will have an impact on the current treatment landscape.
  • A detailed review of the IgG4-RD market, historical and forecasted, is included in the report, covering the 7MM drug outreach.
  • The report provides an edge while developing business strategies by understanding trends shaping and driving the 7MM IgG4-RD market.

IgG4-RD Report Insights

  • Patient population
  • Therapeutic approaches
  • IgG4-RD pipeline analysis
  • IgG4-RD market size and trends
  • Market opportunities
  • Impact of upcoming therapies

IgG4-RD Report Key Strengths

  • Ten years forecast
  • 7MM coverage
  • IgG4-RD epidemiology segmentation
  • Key cross competition
  • Highly analyzed market
  • Drugs uptake

IgG4-RD Report Assessment

  • Current treatment practices
  • Unmet needs
  • Pipeline product profiles
  • Market attractiveness
  • Qualitative analysis (SWOT and conjoint analysis)

FAQs:

  • What was the IgG4-RD market share (%) distribution in 2020, and what would it look like in 2034?
  • What would be the IgG4-RD total market size as well as market size by therapies across the 7MM during the study period (2020-2034)?
  • Which country will have the largest IgG4-RD market size during the study period (2020-2034)?
  • What are the disease risks, burdens, and unmet needs of IgG4-RD?
  • What is the historical IgG4-RD patient pool in the United States, EU4 (Germany, France, Italy, and Spain) and the UK and Japan?
  • What will be the growth opportunities across the 7MM concerning the patient population of IgG4-RD?
  • How many emerging therapies are in the mid-stage and late stage of development for the treatment of IgG4-RD?
  • What are the key collaborations (Industry-Industry, Industry-Academia), Mergers and acquisitions, and licensing activities related to IgG4-RD therapies?
  • What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitations of existing therapies?
  • What are the clinical studies going on for IgG4-RD and their status?
  • What are the key designations that have been granted for the emerging therapies for IgG4-RD?

Reasons to Buy:

  • The report will help in developing business strategies by understanding trends shaping and driving IgG4-RD.
  • To understand the future market competition in the IgG4-RD market and an Insightful review of the SWOT analysis of IgG4-RD.
  • Organize sales and marketing efforts by identifying the best opportunities for IgG4-RD in the US, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan.
  • Identification of strong upcoming players in the market will help in devising strategies that will help in getting ahead of competitors.
  • Organize sales and marketing efforts by identifying the best opportunities for the IgG4-RD market.
  • To understand the future market competition in the IgG4-RD market.

Table of Contents

1. Key Insights

2. Report Introduction

3. Executive Summary

4. Key Events

5. Epidemiology and Market Forecast Methodology

6. IgG4-RD Market Overview at a Glance

  • 6.1. Market Share (%) Distribution by Therapies of IgG4-RD in 2024
  • 6.2. Market Share (%) Distribution by Therapies of IgG4-RD in 2034

7. Disease Background and Overview

  • 7.1. Introduction
  • 7.2. Clinical Phenotype
  • 7.3. Histology Related to IgG4-RD
  • 7.4. Pathogenesis Related to IgG4-RD
  • 7.5. Disease Subtypes and Clinical Manifestations
  • 7.6. Signs and Symptoms Related to IgG4-RD
  • 7.7. Diagnostic Evaluation of IgG4-RD
    • 7.7.1. Biomarkers of Disease Activity and Damage
    • 7.7.2. Differential Diagnosis
    • 7.7.3. Diagnosis Algorithm
  • 7.8. Treatment and Management for IgG4-RD
    • 7.8.1. Treatment and Management Algorithm
  • 7.9. Guidelines, Recommendations, and Management for IgG4-RD
    • 7.9.1. International Consensus Guidance Statement on the Management and Treatment of IgG4-RD (2015)
    • 7.9.2. European Guideline on IgG4-related Digestive Disease-UEG and SFG Evidence-based Recommendations (2020)
    • 7.9.3. The 2020 Revised Comprehensive Diagnostic (RCD) Criteria for IgG4-RD (2021)
    • 7.9.4. American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) Guidelines for Diagnosis (2020)
    • 7.9.5. Current Approach to the Diagnosis of IgG4-RD: A Combination of Comprehensive Diagnostic and Organ-specific Criteria (2017)

8. Epidemiology and Patient Population of IgG4-RD in the 7MM

  • 8.1. Key Findings
  • 8.2. Assumptions and Rationales
  • 8.3. Total Diagnosed Prevalent Cases of IgG4-RD in the 7MM
  • 8.4. The United States
    • 8.4.1. Total Diagnosed Prevalent Cases of IgG4-RD in the United States
    • 8.4.2. Gender-specific Diagnosed Prevalent Cases of IgG4-RD in the United States
    • 8.4.3. Age-specific Diagnosed Prevalent Cases of IgG4-RD in the United States
  • 8.5. EU4 and the UK
    • 8.5.1. Total Diagnosed Prevalent Cases of IgG4-RD in EU4 and the UK
    • 8.5.2. Gender-specific Diagnosed Prevalent Cases of IgG4-RD in EU4 and the UK
    • 8.5.3. Age-specific Diagnosed Prevalent Cases of IgG4-RD in EU4 and the UK
  • 8.6. Japan
    • 8.6.1. Total Diagnosed Prevalent Cases of IgG4-RD in Japan
    • 8.6.2. Gender-specific Diagnosed Prevalent Cases of IgG4-RD in Japan
    • 8.6.3. Age-specific Diagnosed Prevalent Cases of IgG4-RD in Japan

9. Patient Journey

10. Emerging Drugs

  • 10.1. Key Cross Competition
  • 10.2. Obexelimab (XmAb5871): Zenas BioPharma and Bristol Myers Squibb
    • 10.2.1. Product Description
    • 10.2.2. Other Development Activity
    • 10.2.3. Clinical Development
      • 10.2.3.1. Clinical Trial Information
    • 10.2.4. Safety and Efficacy
    • 10.2.5. Analyst Views
  • 10.3. UPLIZNA (inebilizumab): Amgen
    • 10.3.1. Product Description
    • 10.3.2. Other Development Activity
    • 10.3.3. Clinical Development
      • 10.3.3.1. Clinical Trial Information
    • 10.3.4. Safety and Efficacy
    • 10.3.5. Analyst View
  • 10.4. Rilzabrutinib (PRN1008/SAR444671): Sanofi
    • 10.4.1. Product Description
    • 10.4.2. Other Development Activity
    • 10.4.3. Clinical Development
      • 10.4.3.1. Clinical Trial Information
    • 10.4.4. Analyst View

11. IgG4-RD: Seven Major Market Analysis

  • 11.1. Key Findings
  • 11.2. Market Outlook
  • 11.3. Key Market Forecast Assumptions
    • 11.3.1. Cost Assumptions and Rebate
    • 11.3.2. Pricing Trends
    • 11.3.3. Analogue Assessment
    • 11.3.4. Launch Year and Therapy Uptake
  • 11.4. Conjoint Analysis
  • 11.5. Total Market Size of IgG4-RD in the 7MM
  • 11.6. Market Size of IgG4-RD by Therapies in the 7MM
  • 11.7. The United States
    • 11.7.1. Total Market Size of IgG4-RD in the US
    • 11.7.2. Market Size of IgG4-RD by Therapies in the US
  • 11.8. EU4 and the UK
    • 11.8.1. Total Market Size of IgG4-RD in the EU4 and the UK
    • 11.8.2. Market Size of IgG4-RD by Therapies in EU4 and the UK
  • 11.9. Japan
    • 11.9.1. Total Market Size of IgG4-RD in Japan
    • 11.9.2. The Market Size of IgG4-RD by Therapies in Japan

12. Unmet Needs

13. SWOT Analysis

14. KOL Views

15. Market Access and Reimbursement

  • 15.1. United States
    • 15.1.1. Centre for Medicare and Medicaid Services (CMS)
  • 15.2. EU4 and the UK
    • 15.2.1. Germany
    • 15.2.2. France
    • 15.2.3. Italy
    • 15.2.4. Spain
    • 15.2.5. United Kingdom
  • 15.3. Japan
    • 15.3.1. MHLW
  • 15.4. Reimbursement for IgG4-RD

16. Appendix

  • 16.1. Bibliography
  • 16.2. Report Methodology

17. DelveInsight Capabilities

18. Disclaimer

19. About DelveInsight

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