Non-Hodgkin's lymphoma in a woman with adult-onset Still's disease: a case report

INTRODUCTION: Adult onset Still's disease is a chronic multisystemic inflammatory disorder characterized by high spiking fever, polyarthralgia and rash. Lymphadenopathy is a prominent feature of adult onset Still's disease and is seen in about 65% of patients. Searching the medical literature using the MEDLINE database from January 1966 through November 2007 we could only find two reported cases of adult onset Still's disease that had progressed to lymphoma. CASE PRESENTATION: We describe a woman who was diagnosed with adult onset Still's disease and developed lymphoma 10 months after the onset of her symptoms. She initially presented with fever and arthritis of the knees, ankles and shoulders, along with a nonpruritic skin rash, myalgia and weight loss. On physical examination she was found to have several enlarged anterior cervical lymph nodes and left posterior auricular lymph nodes all of which were non-tender, immobile and rubbery. Excisional biopsy of the cervical lymph nodes was negative for malignancy. Bone marrow biopsy was also negative for malignancy. She was treated with prednisone. She remained in good health until she presented 10 months later with low back pain, dyspnea and weight loss. Work up revealed malignant lymphoma. She was treated with chemotherapy and was doing well until she presented with abdominal pain. Work up revealed a cirrhotic liver and ascites. She then passed away from hepatorenal syndrome 13 years after the diagnosis of lymphoma. To our knowledge, this is the third reported case of such an occurrence. CONCLUSION: Although the association between adult onset Still's disease and lymphoma has been rarely reported, careful screening for this malignancy in patients suspected to have adult onset Still's disease is warranted

Recurring Lower Abdominal Pain and Fever as Initial Presentation of Adult Onset Still’s Disease in the Absence of Arthralgia and Other System Involvement

A 34 year-old Afro-Caribbean female presented with recurring episodes of fever and lower abdominal pain over a period of two months not improving despite courses of antibiotics for possible recurrent urinary tract infections. On admission to hospital, patient was treated for a possible pyelonephritis or pelvic inflammatory disease (PID). Extensive investigations into possible source of infection were carried out. However, all of the repeated microbiological cultures were normal. Patient was investigated further for other possible causes including connective tissue disease, haematological disorders, or neoplasm, all of which were normal. Diagnosis of adult onset Still’s disease (AOSD) was confirmed by a rheumatologist based on Yamaguchi’s diagnostic criteria for AOSD alongside significantly raised serum ferritin. Patient was treated with steroids to which she showed remarkable clinical improvement alongside marked reduction in her serum ferritin levels

A promoter haplotype of the interleukin-18 gene is associated with juvenile idiopathic arthritis in the Japanese population

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Still’s-like Disease Induced by Breast Implants in a Middle-Aged Female Health Professional

We report a case of silicone-induced Still’s-like inflammatory syndrome diagnosed after extensive investigation in the department of internal medicine of a secondary hospital. A 45-year-old female healthcare specialist presented with fever, headache, sore throat, myalgia and fatigue. A history of breast augmentation surgery in 2007 was confirmed. Physical examination of the patient was normal while laboratory tests showed a very high white blood cell count. The patient was admitted to hospital due to suspected infection and further laboratory tests were carried out. Inflammatory markers were persistently high despite empirical treatment with doxycycline for possible infection by Rickettsia/Coxiella. As the fever did not resolve and the patient had not improved after more than 3 weeks, the case was investigated as fever of unknown origin. Eventually, the existence of liver granulomas as well as the presence of anti-silicone antibodies confirmed the diagnosis of silicone-induced Still’s-like inflammatory syndrome. In conclusion, this case supports a linkage between silicone breast implants and autoimmune connective tissue diseases

Treatment of adult-onset Still's disease: a review

Adult-onset Still's disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. The former classification, based on the disease course, seems to be quite dated. Indeed, there is accumulating evidence that AOSD can be divided into two distinct phenotypes based on cytokine profile, clinical presentation, and outcome, ie, a "systemic" pattern and an "articular" pattern. The first part of this review deals with the treatments that are currently available for AOSD. We then present the different strategies based on the characteristics of the disease according to clinical presentation. To do so, we focus on the two subsets of the disease. Finally, we discuss the management of life-threatening complications of AOSD, along with the therapeutic options during pregnancy

Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile Still's disease

Background: ‘Persistent pruritic papules and plaques' of Still's disease represents a recently described eruption seen in a subset of patients. Most cases reported in the literature to date have been associated with adult-onset Still's disease. Methods: We present the clinical and histopathologic examinations of three female patients ranging in age from 15 to 54 years. Results: Our three patients each presented with clinical findings consistent with Still's disease. The youngest patient suffered from the juvenile form of Still's disease (systemic-onset juvenile rheumatoid arthritis). Each patient had a persistent, pruritic eruption with histopathologic findings of dyskeratosis confined to the upper layers of the epidermis as well as a sparse superficial dermal infiltrate containing scattered neutrophils. Conclusions: These cases confirm the characteristic clinical and histopathologic findings of ‘persistent papules and plaques of Still's disease’ and show the potential for this eruption in both the adult and juvenile age groups.Fortna RR, Gudjonsson JE, Seidel G, DiCostanzo D, Jacobson M, Kopelman M, Patel RM. Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile Still's disease.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/79183/1/j.1600-0560.2010.01570.x.pd

Summary of Papers Presented at the 45th Annual McGuire Lecture Series

It is the purpose of this presentation to quickly review some of the important points of the papers which were presented at the 45th Annual McGuire Lecture Series on the subject of immunology and rheumatic diseases