Lymph nodes - not lymphoma
Inflammatory disorders (non-infectious)
Adult onset Still's disease (AOSD)

Author: Jaya Balakrishna M.D. (see Authors page)

Revised: 13 July 2017, last major update July 2017

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: Adult onset Still’s disease [title]
Cite this page: Adult onset Still's disease (AOSD). PathologyOutlines.com website. http://pathologyoutlines.com/topic/lymphnodesadultstills.html. Accessed August 20th, 2017.
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-10 coding
  • M061
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
Treatment
  • Mainly anti inflammatory medications, including steroids, NSAIDs and anti rheumatic agents
Gross description
  • Non specific 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
Microscopic (histologic) images

Images hosted on other servers:
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Exuberant pattern of lymphadenopathy

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Various patterns

Positive stains
  • CD20 and CD3 in the follicular and interfollicular areas
  • Interfollicular T cells show CD4 predominance over CD8
  • Immunoblasts are of B cell phenotype with CD30 expression
  • Plasma cells are polyclonal by light chain expression
Molecular / cytogenetics description
  • Polyclonal B and T cell gene rearrangement patterns
Differential diagnosis
Board review question #1
    Which of the following set of markers will be helpful in differentiating a lymph node of a patient with adult onset Still’s disease from angioimmunoblastic T cell lymphoma?

  1. CD20, CD3, CD45
  2. CD3, CD4, CD8
  3. CD30, CD15, OCT2
  4. PD1, BCL6, CD10
Board review 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.