Cyclosporine A-induced neck fibrosis in a patient with adult-onset Still’s disease

Cyclosporine A (CsA) is an immunosuppressive agent used for the prevention of graft rejection during organ and bone marrow transplantation. CsA is also used for the treatment of various inflammatory rheumatic diseases. Although different side effect profiles have been reported, nephrotoxicity, renal vascular damage, hypertension, and gingival hypertrophy are among the most commonly encountered side effects. The development of massive fibrosis in the neck associated with CsA in a 30-year-old male patient with Still’s disease is presented herein. Significant regression was observed after the discontinuation of CsA.     

Encephalic large arteries narrowness and peripheral neuropathy in a patient with adult-onset Still’s disease

Adult-onset Still's disease (AOSD) is a rare, systemic inflammatory disorder, characterized by spiking high fever, fever-associated evanescent rash, arthritis, myalgia, serositis and hepatosplenomegaly. White blood cell count, neutrophilic cell count, and serum ferritin level are markedly elevated in the active stage of the disease. Neurological complications of AOSD commonly were cranial nerve palsies, seizures, aseptic meningoencephalitis, peripheral neuropathy and Miller-Fisher syndrome. We report a previously healthy 60-year-old Chinese man who fulfilled the criteria for AOSD and had a combination of focal and peripheral neurological symptoms. Magnetic resonance angiography (MRA) and transcranial Doppler ultrasonography (TCD) showed narrowness of cerebral blood vessel. Peripheral neuropathy was confirmed by electromyography and sural nerve biopsy. His generalized neuropathy and other symptoms were rapidly improved by receiving glucocorticoid therapy. We do a literature review about neurological manifestations observed in AOSD patients.     

Crohn’s disease complicated by adult-onset Still’s disease

A 31-year-old man with Crohns disease developed arthritis, spiking fever, and skin rash indistinguishable from that of adult-onset Stills disease. He was admitted to our hospital because of a periumbilical intestinal skin fistula. Crohns disease had been diagnosed in 1991, and had required intestinal resection twice, and schizophrenia had been diagnosed in 1993. He developed polyarthritis and spiking fever, accompanied by a macular skin rash on both forearms. Marked hepatosplenomegaly and bilateral pleural effusion were detected on computed tomography examination. These findings are indistinguishable from those of adult-onset Stills disease. Because his mental status had deteriorated following high-dose prednisolone on a previous admission, he was treated with an immunosuppressive agent on this occasion, with the treatment being successful. This is the first report of adult-onset Stills disease complicating Crohns disease. In patients with Crohns disease, polyarthritis and skin rash can easily be misdiagnosed as enteropathic arthritis with erythema nodosum associated with the Crohns disease. Although adult-onset Stills disease may not be fatal, early diagnosis is important because it can, in rare cases, result in life-threatening complications.     

A case of paraneoplastic syndrome mimicking adult-onset Still’s disease

A 49-year-old woman was admitted to our hospital because of fever of unknown origin. The patient had long-lasting spiking fever, hepatosplenomegaly, pleural effusion, and skin rash. Laboratory tests showed marked leukocytosis and an extremely high serum ferritin level (240000ng/ml) accompanied by disseminated intravascular coagulation and hemophagocytic syndrome. Most of the patients features were compatible with a diagnosis of adult-onset Stills disease (AOSD), the rash, however, was not a typical rheumatoid rash but multiforme erythema. Biopsy of a breast nodule revealed breast cancer, leading us to a diagnosis of paraneoplastic syndrome mimicking AOSD. Although this is a rare disorder, cases resembling the present one have been reported, indicating the importance of including paraneoplastic syndrome in the differential diagnosis of AOSD.     

A case of paraneoplastic syndrome mimicking adult-onset Still’s disease

Abstract

A 49-year-old woman was admitted to our hospital because of fever of unknown origin. The patient had long-lasting spiking fever, hepatosplenomegaly, pleural effusion, and skin rash. Laboratory tests showed marked leukocytosis and an extremely high serum ferritin level (240?000?ng/ml) accompanied by disseminated intravascular coagulation and hemophagocytic syndrome. Most of the patient’s features were compatible with a diagnosis of adult-onset Still’s disease (AOSD), the rash, however, was not a typical rheumatoid rash but multiforme erythema. Biopsy of a breast nodule revealed breast cancer, leading us to a diagnosis of paraneoplastic syndrome mimicking AOSD. Although this is a rare disorder, cases resembling the present one have been reported, indicating the importance of including paraneoplastic syndrome in the differential diagnosis of AOSD.     

Severe hyperferritinemia—a clue for severe hepatitis in a patient with adult-onset Still’s disease

Clinical Rheumatology - A 19-year old previously healthy man developed the adult-onset Still’s disease (AOSD) with high ferritin levels. Corticosteroids induced clinical remission with...     

Therapeutic responses and prognosis in adult-onset Still’s disease

To date, the treatment of adult-onset Still’s disease (AOSD) has been largely empirical; therefore, this study was conducted to investigate the response to therapy and prognostic factors of AOSD. Fifty-four Korean patients with AOSD were enrolled based on Yamaguchi’s criteria. We retrospectively analyzed the treatments and prognosis. Thirty-nine patients (72.2%) were female, and the average age at disease onset was 37.3 years. Twenty-nine patients had a monocyclic disease (53.7%), five had a polycyclic (9.3%) and fifteen had a chronic articular disease (27.7%) and five died (9.3%). The elevated ESR and corticosteroids refractoriness were associated with poor prognosis (P = 0.023 and P = 0.009, respectively). The patients that died were older than those survived (49.2 ± 11.8 vs. 42.2 ± 14 year old, P = 0.024). Forty-two patients were treated with non-steroidal anti-inflammatory drugs; however, they also needed corticosteroids and intravenous immunoglobulin (IVIG). Among 50 patients treated with high-dose corticosteroids, 21 patients (42%) were resistant to corticosteroids and treated with IVIG or anti-tumor necrosis factor (TNF) agents. Of the 23 patients medicated with IVIG, the prognosis was better in IVIG-responsive patients, indicating a therapeutic effect. Methotrexate was the most commonly used disease modifying anti-rheumatic drugs (27 patients, 50%), and the corticosteroid requirements were lower in the methotrexate-responsive patients. Approximately half of AOSD patients had a poor prognosis and were corticosteroids resistance. An elevated ESR and non-response to corticosteroids were associated with poor prognosis. Patients who died were older than those survived.     

A case of uveitis in adult-onset Still’s disease with ophthalmologic symptoms

Adult-onset Still’s disease (AOSD) is a rare and systemic inflammatory disorder of unknown etiology and pathogenesis. AOSD is characterized by high fever accompanied by a range of systemic symptoms. However, there are rare cases of AOSD with ophthalmologic symptoms as well as with an obvious causation of corticosteroid withdrawal. In this case, a 43-year-old male patient diagnosed with AOSD showed ocular inflammation after withdrawing from corticosteroid treatment. This patient was treated with prednisolone for AOSD and discharged after achieving complete remission of breathlessness, backache, thoracalgia, joint pain, and spiking fever. The patient unauthorizedly stopped taking prednisolone after he was discharged from the hospital and returned to the Department of Ophthalmology with the complaint of decreased visual acuity in both eyes for half a month and sudden vision loss in the left eye for 3 days. After regular ophthalmologic examinations and fluorescence angiography examination, he was diagnosed with acute panuveitis as the manifestation of AOSD. Uveitis was effectively treated with corticosteroid drugs. This case reported a rare manifestation of AOSD in an ophthalmological system that was associated with the withdrawal of corticosteroid treatment. This report highlighted the therapeutic effect of local and systemic corticosteroid use for AOSD manifested with uveitis. This case is interesting for both rheumatologists and ophthalmologists.     

Macrophage activation syndrome associated with adult-onset Still’s disease: a multicenter retrospective analysis

Clinical Rheumatology - To explore the clinical features, treatments, and prognostic factors of adult-onset Still’s disease (AOSD)-associated macrophage activation syndrome (MAS), we...     

Fulminant myocarditis with adult-onset Still’s disease: case-based review

Clinical Rheumatology - Myocarditis has been reported as a life-threatening complication of adult-onset Still’s disease (AOSD), but fulminant myocarditis with AOSD is very rare. We hereby...     

HLA-DQB1 DPB1 alleles in Japanese patients with adult-onset Still’s disease

Abstract

Objective: HLA class II alleles are major determinants of genetic predisposition to rheumatic diseases. Predisposing effects of HLA had been suggested in AOSD, however, ethnic differences may account for variations in AOSD association with HLA. We determined the contribution of HLA-DQB1, DPB1 alleles to susceptibility to Adult-onset Still’s disease (AOSD) in the Japanese population.

Methods: HLA-DQB1 and DPB1 alleles were analyzed in 87 Japanese patients with AOSD and 413 Japanese healthy subjects.

Results: We found significant association between HLA-DQB1*06:02 (Pc?=?0.010, odds ratio: 2.54) and AOSD, whereas there was no association between the DQB1*06:02 allele and disease phenotypes of AOSD. Moreover, we did not find a predisposing effect of the HLA-DPB1 allele to AOSD. Haplotype analysis showed that presence of DRB1*15:01–DQB1*06:02 was associated with Japanese patients with AOSD. However, conditional logistic regression tests were unable to demonstrate independent association between DRB1*1501 or DQB1*0602 and AOSD.

Conclusions: Our results show significant association between AOSD and the HLA DQB1*06:02 allele, and between the DRB1*1501–DQB1*06:02 haplotype and AOSD susceptibility. These findings suggest that genetic susceptibility to AOSD depends on the genotype combinations of HLA DRB1 and DQB1 alleles.     

Lymphadenopathy in adult-onset Still’s disease mimicking peripheral T-cell lymphoma

Lymphadenopathy (LAP) that is seen in adult onset Stills disease (AOSD) may be confused with lymphoma. Here we present a patient with AOSD and with LAP that histopathologically mimicked T-cell lymphoma.Abbreviations AOSD Adult-onset Stills disease - CRP C-reactive protein - ESR Erythrocyte sedimentation rate - LAP Lymphadenopathy - TGGE Temperature gradient gel electrophoresis     

Bilateral low lobar atelectasis in a young woman with adult-onset Still’s disease

Adult-onset Still’s disease (AOSD) is an uncommon inflammatory condition of unknown origin and pathogenesis. Pulmonary manifestations are rare and include pleuritis and transient radiological infiltrations. We report a case of a young woman with AOSD who developed unusual respiratory symptoms, with bilateral lower lobar atelectasis and restrictive syndrome and reviewed the literature on it. We illustrate the difficulties in diagnosis of atypical pulmonary defect with unusual radiological aspects and discuss causality relationship between lung abnormalities and Still’s disease.     

Amyloid goiter as the first clinical sign of systemic amyloidosis in a patient with adult-onset Still’s disease

Abstract

We present a patient with amyloid goiter due to secondary amyloidosis which resulted from adult-onset Still’s disease. Amyloid goiter occurs as one of the rare manifestations of systemic amyloidosis. In our case the initial manifestation of amyloidosis was a fast growing goiter without other clinical abnormalities, which is unusual in the context of previously reported cases.     

Marked effect and steroid-sparing ability of anakinra on a patient with refractory adult-onset Still’s disease

We report a case of refractory adult-onset Still’s disease (AOSD) successfully controlled with anakinra, an interleukin-1 (IL-1) receptor antagonist. The patient was a 23-year-old Japanese woman with AOSD who could not be induced into remission despite of two courses of pulsed methylprednisolone followed by high-dose glucocorticoid administration in conjunction with high-dose intravenously administered γ-globulin and methotrexate. To the best of our knowledge, this is the first case report in Japan of AOSD remission induced with anakinra.     

Elevated serum levels of interleukin-10 in adult-onset Still’s disease are associated with disease activity

Clinical Rheumatology - To evaluate the serum levels of the anti-inflammatory cytokine interleukin-10 (IL-10) in patients with adult-onset Still’s disease (AOSD), a rare, systemic, and...