Lymph nodes & spleen, nonlymphoma
Lymph node-nonspecific findings
Adult onset Still disease
Author: Jayalakshmi Balakrishna, M.D.
Last author update: 1 July 2017
Last staff update: 12 May 2021
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: Adult onset Still disease[TI]
Table of Contents
Definition / general | Essential features | ICD coding | Epidemiology | Sites | Pathophysiology | Clinical features | Diagnosis | Laboratory | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Positive stains | Molecular / cytogenetics description | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1Cite this page: Balakrishna J. Adult onset Still disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphnodesadultstills.html. Accessed June 4th, 2023.
Definition / general
- Rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema and arthralgia, accompanied frequently by lymphadenopathy
Essential features
- Fever, skin rash, joint pains, lymphadenopathy with paracortical hyperplasia and immunoblastic reaction
ICD coding
Epidemiology
- Rare, shows female predilection and more common in young adults
Sites
- Systemic disease
- Generalized lymphadenopathy
Pathophysiology
- Pathophysiology is yet to be clearly defined
- Interleukins, macrophage colony stimulating factor, interferon gamma and tumor necrosis factor alpha may play a role
- May also be due to genetic factors, immune dysfunction and infections
Clinical features
- Systemic inflammatory disease manifesting with spiking fever, sore throat, arthralgia, skin rash and hepatosplenomegaly
- Reactive hemophagocytic syndrome is also reported
Diagnosis
- Clinical, laboratory and imaging results
Laboratory
- Neutrophilic leukocytosis, anemia, elevated ferritin, C reactive protein (CRP), erythrocyte sedimentation rate (ESR) and abnormal liver function tests (AST and ALT)
Radiology description
- Affected joints show progressive erosions and narrowing joint space
- Commonly associated with hepatosplenomegaly
Prognostic factors
- Disease can be self limiting and the prognosis depends on the specific disease pattern
- Overall, patients with only localized disease have a better prognosis compared to those with more disseminated disabilities or severe complications
- Disease can be monocyclic - self limiting, polycyclic - symptoms recur or have chronic articular pattern
Case reports
- 28 year old woman with AOSD (Acta Med Iran 2016;54:683)
- 53 year old woman diagnosed with AOSD based on Yamaguchi criteria (Am J Case Rep 2017;18:119)
- Persistent pruritic lesions in adult onset Still disease (Am J Med Sci 2016;352:540)
- Three Japanese women ages 22, 26 and 63 with lymph node lesions from AOSD (Int J Surg Pathol 2002;10:197)
Treatment
- Mainly antiinflammatory medications, including steroids, NSAIDs and antirheumatic agents
Gross description
- Nonspecific enlargement of lymph nodes, usually small size
Microscopic (histologic) description
- Major histological findings limited to paracortical hyperplasia or a mixed pattern with paracortical and diffuse or paracortical, follicular and diffuse hyperplasia, with or without sinus histiocytosis
- Vascular proliferation in the interfollicular areas, immunoblastic reaction and mixed inflammatory cell infiltrations are also described commonly
- Rare findings described include pericapsular endarteritis and hemophagocytic features
Positive stains
Molecular / cytogenetics description
- Polyclonal B and T cell gene rearrangement patterns
Differential diagnosis
Additional references
Board review style question #1
- Which of the following set of markers will be helpful in differentiating a lymph node of a patient with adult onset Still disease from angioimmunoblastic T cell lymphoma?
- CD20, CD3, CD45
- CD3, CD4, CD8
- CD30, CD15, OCT2
- PD1, BCL6, CD10
Board review style answer #1
D. PD1, BCL6, CD10 - markers for follicular helper T cell phenotype. Angioimmunoblastic T cell lymphoma is of follicular helper T cell origin and is positive for PD1, BCL6 and CD10.
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Reference: Adult onset Still disease
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Reference: Adult onset Still disease
