Adult‐onset still's disease

Cush, John J.; Medsger, Thomas A.; Christy, Wallace C.; Herbert, D C; Cooperstein, Lawrence A.

doi:10.1002/art.1780300209

Cited by 301 publications

(95 citation statements)

References 16 publications

(6 reference statements)

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“…About 41% of patients had an inter-articular space reduction of the wrist, carpal and carpometacarpal joints [22]. Joint destruction interests particularly hips and knees in some cases and requires the establishment of a total prosthesis; non-erosive ankyloses of carpo-metacarpal and inter-carpal joints appear after a few years in AOSD [21] [23].…”

Section: Discussionmentioning

confidence: 99%

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Adult Onset Still’s Disease: Articular Manifestations in Twenty Cases

Mahfoudhi¹,

Gorsane²,

Battikh³

et al. 2015

OJCD

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The adult onset Still's disease is a rare inflammatory pathology of unknown pathogeny. The clinical features are variable. The diagnosis is difficult since exclusion of infectious, systemic and tumoral pathologies should be done. The articular complications are frequent and can be revelatory of this pathology. The articular prognosis depends on the diagnosis delay and the treatment efficiency. Our study aims to analyze different aspects of articular manifestations complicating adult onset Still's disease to define epidemiological, clinical and evolving characteristics of these complications. It was a cross-sectional study concerning 20 cases of adult onset Still's disease diagnosed from 1990 to 2015 in the internal medicine A department of Charles Nicolle Hospital in Tunis, meeting Yamaguchi criteria. We identified clinical, radiological, evolving and therapeutic profile of the articular manifestations occurred in these patients. There were 13 women and 7 men. The average age was 25 years. The arthralgias were reported in all cases; while, the arthritis interested fifteen patients. A hand deformation was found in four patients. A wrist ankylosis was noted in one case and a flexion elbow in one patient. The standard articular radiographs were normal in twelve cases. The treatment associated essentially non-steroidal anti-inflammatory and/or corticosteroids and/or methotrexate. Concerning the evolving profile, the monocyclic form was pre-sent in 25% of the cases, the intermittent form in 45% and the chronic articular form in 30% of our patients. The adult onset Still's disease is rare and heterogeneous. The articular disturbances are frequent and have various outcomes.

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Section: Discussionmentioning

confidence: 99%

“…According to the literature, NSAIDs did not allow resolution of symptoms in 20% of cases. The efficiency of corticosteroids in the AOSD observations was spectacular in the majority of cases [8] [23].…”

Section: Discussionmentioning

confidence: 99%

Adult Onset Still’s Disease: Articular Manifestations in Twenty Cases

Mahfoudhi¹,

Gorsane²,

Battikh³

et al. 2015

OJCD

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“…Serosal involvement may occur in the setting of systemic inflammatory disorders such as adult-onset Still's disease (AOSD) [12] , an entity in which laboratory testing and histological features are nonspecific. Accordingly, its diagnosis should rely on clinical suspicion and is often supported by the use of classification criteria [12,13] .…”

Section: Consanguinity Of Parentsmentioning

confidence: 99%

“…Accordingly, its diagnosis should rely on clinical suspicion and is often supported by the use of classification criteria [12,13] . The classic presentation of AOSD includes fever, a 'salmon-pink' rash, arthralgia, arthritis, and lymphadenopathy while serositis and other atypical manifestations are seldom reported and are considered not to be major diagnostic criteria [12][13][14][15] .…”

Section: Consanguinity Of Parentsmentioning

confidence: 99%

Idiopathic chronic polyserositis with massive ascites responding to colchicine in an adult: A case report

Fernández-Ayala

Carrascosa

Salcines-Caviedes

2014

CRIM

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Objective: While there are many different causes of ascites, most cases are due to cirrhosis. However, a serum/ascites albumin gradient <1.1 indicates that the ascites is not induced by portal hypertension and mandates a search for other etiologies, including causes of serositis. We describe a 70-year-old patient with persistent ascites and pleuropericarditis who underwent a successful outcome while on colchicine treatment. Methods:This study was delineated on a case-report basis. Besides supportive care measures, we performed a comprehensive clinical and complementary assessment of the patient, which included laboratory tests, imaging techniques, endoscopic evaluation, full microbiological and pathological studies, diagnostic laparoscopy and thoracoscopy, and genetic testing for autoinflammatory diseases. Results:Diagnostic workup failed to demonstrate the presence of an underlying etiologic disorder. Large-volume paracentesis was performed once monthly during 13 consecutive months due to painless, massive accumulation of peritoneal fluid. Eventually, colchicine therapy induced a dramatic response with clinical and radiological disappearance of polyserositis within three months of starting on the drug. While on colchicine, the disease has not recurred throughout a thirty two months period of follow-up so far. To our knowledge, the occurrence of idiopathic, chronic refractory ascites and pleuropericarditis resolved under colchicine treatment in an adult not diagnosed with Familial Mediterranean Fever is an exceptional event.Conclusion: We suggest that in adult patients suffering from idiopathic chronic polyserositis including massive ascites the possible existence of an underlying autoinflammatory condition should be sought. In such a case, a colchicine trial might be regarded as an early suitable therapeutic option.

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“…The classic triad includes quotidian (daily) spiking fevers (≥39°C), juvenile idiopathic arthritis or rheumatoid rash, and polyarthritis. 13,14 The cutaneous features associated with AOSD include pruritis, urticaria (<40%), and salmonpink evanescent (changes day to day) rash usually on the trunk, neck, and extremities and almost never on the face, palms, or soles. Other characteristic manifestations include a prodromal sore throat (in adults, not children), arthralgias, myalgias, rapid weight loss, serositis (pleuritis or pericarditis), generalized lymphadenopathy, hepatomegaly (often with elevated hepatic enzymes), and splenomegaly.…”

Section: Still Diseasementioning

confidence: 99%

Autoinflammatory Syndromes

Pediatric Rheumatology in Clinical Practice

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Adult‐onset still's disease

Cited by 301 publications

References 16 publications

Adult Onset Still’s Disease: Articular Manifestations in Twenty Cases

Adult Onset Still’s Disease: Articular Manifestations in Twenty Cases

Idiopathic chronic polyserositis with massive ascites responding to colchicine in an adult: A case report

Autoinflammatory Syndromes

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