Department of Histopathology, Panjab University, A.B. Diagnostics, India
Adult onset Still’s disease emerges as an infrequently discerned, systemic inflammatory disease classically delineating a triad of clinical symptoms as pyrexia with spikes, maculopapular exanthema and arthralgia. The systemic disease demonstrating generalized lymphade- nopathy frequently expounds paracortical hyperplasia and immunoblastic reaction. The exceptionally encountered disorder commonly implicates young adults and exemplifies a female predilection [1,2]. Of obscure pathophysiology, adult onset Still’s disease is associated with secretion of interleukins, macrophage colony stimulating factor (M-CSF), interferon gamma and tumour necrosis factor alpha. Besides, genetic factors, immune dysfunction and diverse infections may engender the disorder [3,4]. Adult onset Still’s disease emerges as a systemic inflammatory disease expounding clinical symptoms as pyrexia with spikes, sore throat, arthralgia, cutaneous rash and hepatosplenomegaly. Additionally, reactive haemophagocytic syndrome may be observed [3,4]. Grossly, the condition is accompanied by a nonspecific enlargement of involved, miniature lymph nodes [4,5]. Upon microscopy, paracortical region expresses predominant hyperplasia. Alternatively, a ‘mixed’ pattern with paracortical and diffuse or paracortical, follicular and diffuse hyperplasia of lymphoid architecture is observed. Sinus histiocytosis may or may not concur. Vascular proliferation appears confined to inter-follicular areas. Frequently, immunoblastic reaction and mixed inflammatory cell infiltration may be observed. Exceptionally, pericapsular endarteritis or features of haemophagocytosis may appear [4,5]. Adult onset Still’s disease demonstrates immune reactivity to CD20 and CD3, especially within follicular and inter-follicular areas. Inter-follicular T cells appear preponderantly immune reactive to CD4, in contrast to CD8. Constituent immunoblasts delineate a B cell phenotype with immune-expression of CD30. Plasma cells expound a polyclonal light chain expression. Adult onset Still’s disease demonstrates a polyclonal profile with B and T cell genetic rearrangements [8,9]. Adult onset Still’s disease requires segregation from neoplasms as peripheral T cell lymphoma (PTCL), angioimmunoblastic T cell lym- phoma (AITL) and T zone variant of peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) [8,9]. Adult onset Still’s disease may be appropriately ascertained by cogent clinical, haematological and biochemical parameters. Concurrence with adjunct imaging features appear beneficial [8,9]. Subjects depict anaemia, neutrophilic leucocytosis and elevated erythrocyte sedimentation rate (ESR). Besides, elevated serum levels of ferritin and C reactive protein (CRP) are discerned. Liver function tests are abnormal with augmented levels of serum aspartate amino- transferase (AST) and alanine aminotransferase (ALT) [9,10]. Upon radiography, involved joints delineate progressive bony erosion and narrowing of joint spaces. Frequently, the condition concurs with hepatosplenomegaly [9,10]. Adult onset Still’s disease may be suitably managed with anti-inflammatory drugs or non steroidal anti-inflammatory drugs (NSAIDs), corticosteroids or antirheumatic agents [10,11]. Adult onset Still’s disease is a self limiting condition wherein prognostic outcomes are contingent to specific disease pattern. Generally, subjects with localized disease display a superior prognosis, in contrast to individuals with disseminated disabilities or severe complica- tions. The disease can be self limiting and monocyclic or polycyclic wherein clinical symptoms appear to reoccur or demonstrate a chronic, articular pattern [10,11].
Anubha Bajaj. “Symbolic and Indicatory-Adult Onset Still’s Disease”. EC Microbiology 22.10 (2026): 01-04.
© 2026 Anubha Bajaj. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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