Immunology

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Hemophagocytic lymphohistiocytosis (HLH)

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder characterized by pathologic immune activation and systemic hyperinflammation.

The inherited form of HLH is known as primary hemophagocytic lymphohistiocytosis (pHLH). pHLH is a rare disorder associated with impaired cytotoxic function of NK cells and CD8+ T cells. It typically manifests during infancy and is invariably fatal if untreated.

Other forms of HLH are known as secondary HLH, including a subset referred to as macrophage activation syndrome (MAS). HLH/MAS is a rare, potentially fatal complication of rheumatological conditions, especially systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), that can result in significant morbidity to both pediatric and adult patients.

HLH publications

The publications on this page are sponsored by Sobi and pertain to select Sobi products in this disease area. This page is not intended to serve as a comprehensive overview of all publications in this disease area. Publications may include information for which the product is not approved and/or that is inconsistent with the product uses described in the Prescribing Information. Sobi does not suggest or recommend the use of Sobi products in any manner other than as described in the Prescribing Information.

Emapalumab in Patients With Macrophage Activation Syndrome Associated With Still's Disease: A Population Pharmacokinetic/​Pharmacodynamic Analysis
Clinical and Translational Science, February 2025
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Emapalumab Treatment in Patients With Rheumatologic Disease–Associated Hemophagocytic Lymphohistiocytosis in the United States: A Retrospective Medical Chart Review Study
Arthritis & Rheumatology, February 2025
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Real-World Treatment Patterns and Outcomes in Patients With Primary Hemophagocytic Lymphohistiocytosis Treated With Emapalumab
Blood Advances, May 2024
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Exposure-Safety Relationship for Patients With Primary Hemophagocytic Lymphohistiocytosis Treated With Emapalumab
Pediatric Blood & Cancer, November 2023
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Emapalumab in Children With Primary Hemophagocytic Lymphohistiocytosis
The New England Journal of Medicine, May 2020
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HLH resources

Review the recommended resources for additional clinical insights.

MAS Mechanism of Disease

View the infographic to learn more about the role of IFNg in MAS pathophysiology.

Key Biomarkers in MAS

View the infographic to learn more about the latest biomarkers for MAS management.

MAS Epidemiology and Clinical Diagnosis

View the infographic to learn more about the diagnosis of MAS.

MAS Guidelines

View the infographic to learn more about treatment aims and considerations in MAS management.

Neonatal-onset multisystem inflammatory disease (NOMID)

NOMID is a rare, predominantly pediatric autoinflammatory disease that can be challenging to diagnose and can cause long-term disability without treatment. Globally, only 100 people have been reported to have NOMID.

NOMID publications

The publications on this page are sponsored by Sobi and pertain to select Sobi products in this disease area. This page is not intended to serve as a comprehensive overview of all publications in this disease area. Publications may include information for which the product is not approved and/or that is inconsistent with the product uses described in the Prescribing Information. Sobi does not suggest or recommend the use of Sobi products in any manner other than as described in the Prescribing Information.

Sustained Response and Prevention of Damage Progression in Patients With Neonatal-Onset Multisystem Inflammatory Disease Treated With Anakinra: A Cohort Study to Determine Three- and Five-Year Outcomes
Arthritis & Rheumatism, July 2012
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NOMID resources

Review the recommended resources for additional clinical insights.

The Role of IL-1 in Autoinflammation

View the presentation to learn more about the local and systemic effects of IL-1.

Pathophysiology of IL-1–Triggered Diseases

View the infographic to learn more about clinical signs and symptoms, testing, and biomarkers.

Deficiency of the interleukin-1 receptor antagonist (DIRA)

DIRA is an ultra-rare, autosomal recessive disorder caused by mutations in the IL1RN gene, leading to a loss of production and function of IL-1Ra. DIRA generally presents at or near birth and can escalate to life-threatening inflammation. To date, DIRA has been identified in fewer than 50 people worldwide.

DIRA publications and case reports

The publications on this page are sponsored by Sobi and pertain to select Sobi products in this disease area. This page is not intended to serve as a comprehensive overview of all publications in this disease area. Publications may include information for which the product is not approved and/or that is inconsistent with the product uses described in the Prescribing Information. Sobi does not suggest or recommend the use of Sobi products in any manner other than as described in the Prescribing Information.

Deficiency of Interleukin-1 Receptor Antagonist (DIRA): Report of the First Indian Patient and a Novel Deletion Affecting IL1RN
Journal of Clinical Immunology, July 2017
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DIRA resources

Review the recommended resources for additional clinical insights.

The Role of IL-1 in Autoinflammation

View the infographic to learn more about the local and systemic effects of IL-1.

Diagnosis of IL-1–Driven Diseases

View the presentation to learn more about clinical signs and symptoms, testing, and biomarkers.

Hereditary tyrosinemia type 1 (HT-1)

HT-1 is a rare, autosomal recessive genetic metabolic disorder with a global birth incidence of 1/100,000 in most areas. HT-1 is caused by a deficiency in the enzyme fumarylacetoacetate hydrolase (FAH), which is essential for the final breakdown of the amino acid tyrosine. Without proper breakdown, tyrosine and its metabolites build up in the liver, potentially resulting in severe liver disease. Tyrosine may also accumulate in the kidneys and central nervous system.

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