Adult-onset Still disease (AOSD), commonly referred in the literature as the adult form of systemic-onset juvenile idiopathic arthritis, is a disorder of unknown etiology [1] [2]. The clinical presentation might be quite different from patient to patient, but the following manifestations are included in the diagnostic criteria [1] [3] [4] [5]:

• Fever - Although not specific, the recurring appearance of very high fever (> 39°C) that lasts for a few hours and mainly happens in the late afternoon or early evening period, is a hallmark of AOSD [3] [6].
• Joint pain - The involvement of knees, wrists, or ankles is another important manifestation of AOSD [3] [4]. Arthritis usually accompanies fever and has a relatively short duration as well [3].
• Rash - A salmon-pink or erythematous nonpruritic maculopapular rash, developing together with fever and joint pain, is frequently observed, and is most commonly seen on the trunk and the proximal limbs [5].

Other common manifestations of AOSD are lymphadenopathy, hepatosplenomegaly, muscle aches, and pharyngitis [1] [7]. The clinical course of the disorder can be highly variable, ranging from mild and self-limiting to severely debilitating disease [3]. Furthermore, AOSD may cause a myriad of complications in affected individuals, such as pulmonary hypertension, thrombotic thrombocytopenic purpura (TTP), amyloidosis, and macrophage activation syndrome [1]. An early detection of this disorder could significantly aid in successful treatment and improvement of the overall quality of life.

• Splenomegaly

  – Elevated acute-phase reactants: elevated ESR, CRP, white count, and hyperferritinemia (69-97% of patients) -Other findings that may be found include: myalgia, sore throat, hepatomegaly, splenomegaly, lymphadenopathy, serositis, abdominal pain, elevated [utswim.wordpress.com]

  Adult-onset Still disease (AOSD) is a systemic inflammatory disorder characterized clinically by high spiking fever, polyarthralgia/arthritis, a salmon-pink evanescent rash, predominantly neutrophilic leucocytosis, lymphadenopathy, liver dysfunction, and splenomegaly [ncbi.nlm.nih.gov]

• Generalized Lymphadenopathy

  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 [pathologyoutlines.com]

  It differs from classical rheumatoid arthritis in that it is more often marked by acute febrile onset, and generalized lymphadenopathy and hepatosplenomegaly are more prominent. [icd10data.com]

  Other characteristic manifestations include a prodromal sore throat, arthralgias, myalgias, weight loss, serositis, hepatomegaly (often with elevated hepatic enzymes), splenomegaly and generalized lymphadenopathy. [rheumnow.com]

  B (Minor criteria) comprises (i) TLC > or = 15000/mm 3 (ii) Still's rash (iii) Pleuritis or pericarditis (iv) Hepatomegaly or splenomegaly or generalized lymphadenopathy. Our patient qualified for all criteria from A and two criteria from B. [ijnm.in]

  On examination, she looked pale, febrile (temperature 39.2ºC), had non-suppurative pharyngitis and generalized lymphadenopathy. The lymph nodes were slightly tender, small and discrete. [pjms.com.pk]

• Fever

  Presentation: – Fever: nearly always present (85-100%). Quotidian or twice-daily. Usually in the PM and first sign of disease – Rash: occurs in 62-77% of patients. Evanescent rash that occurs during fever spikes. Macular and papular. [utswim.wordpress.com]

  Arthritis usually accompanies fever and has a relatively short duration as well. [symptoma.com]

  We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. [ncbi.nlm.nih.gov]

  Fevers develop each day (usually in the afternoon or evening), and the rash comes and goes with the fever. Other signs and symptoms may include abdominal pain, pleurisy, swollen lymph nodes, and weight loss. [rarediseases.info.nih.gov]

• Lymphadenopathy

  Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy. [symptoma.com]

  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 [pathologyoutlines.com]

  – Elevated acute-phase reactants: elevated ESR, CRP, white count, and hyperferritinemia (69-97% of patients) -Other findings that may be found include: myalgia, sore throat, hepatomegaly, splenomegaly, lymphadenopathy, serositis, abdominal pain, elevated [utswim.wordpress.com]

  A 33 year old Hispanic male presented with recurrent daily fevers, sore throat and lymphadenopathy. Diagnostic evaluation including lymph node biopsy was negative for infectious causes. [oatext.com]

  Adult onset Still's disease (AOSD) is a systemic inflammatory disease characterized by high-fevers, articular involvement, maculopapular rash, hepatosplenomegaly, lymphadenopathy, and a neutrophilic leukocytosis. [ncbi.nlm.nih.gov]

• High Fever

  A 48-year-old woman was admitted because of spiking high fever, sore throat, and jaundice. [ncbi.nlm.nih.gov]

  Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy. [symptoma.com]

  Major diagnostic criteria include arthralgia for more than 2 weeks, intermittent high fever for more than 1 week, characteristic rash, and white blood cell count above 10,000. [orpha.net]

• Anemia

  Autoimmune hemolytic anemia. Nihon Rinsho 2008;66:520–523. 15. Pirofsky B. Clinical aspects of autoimmune hemolytic anemia. Semin Hematol 1976;13:251–265. [synapse.koreamed.org]

  AOSD patients with RHS showed higher frequencies of leukopenia, anemia, thrombocytopenia, hypoalbuminemia, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia, and elevated lactate dehydrogenase levels than did those without RHS. [ncbi.nlm.nih.gov]

  METHODS: Previously we developed a method to measure the iron content of ferritin; we investigated the usefulness of this method to establish the iron status of patients with anemia of inflammation. [jrheum.org]

  CC: 6 days of fevers and URI symptoms ID: 67 yo female with pernicious anemia presents with 6 days of fevers and URI symptoms and 3 weeks of nonpruritic, round, scaly rash on her forearm. [uscmedicine.blog]

• Collapse

  Renal biopsy: collapsing glomerulopathy. Arrows: open glomerular loops; arrow heads: collapsed glomerular loops. F ig. 3. Renal biopsy: collapsing glomerulopathy. Arrows: open glomerular loops; arrow heads: collapsed glomerular loops. [rheumatology.oxfordjournals.org]

  The clinico-biological picture of AOSD usually includes high spiking fever with joint symptoms, evanescent skin rash, sore throat, striking neutrophilic leukocytosis, hyperferritinemia with collapsed glycosylated ferritin (<20%), and abnormal liver function [ncbi.nlm.nih.gov]

  Postmortem biopsy of the liver showed central necrosis with collapse of reticulin structure. Discussion Still’s disease is an uncommon rheumatic condition of unknown aetiology with recognised increased frequency in adults. [tropicalgastro.com]

  Dupont, Adult-onset Still's disease associated with collapsing glomerulopathy, Clinical Kidney Journal, 3, 1, (54), (2010). [doi.org]

• Pharyngitis

  Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy. [symptoma.com]

  Arthritis (OR, 8.6; 95% confidence interval [CI], 1.5-49.1; p = 0.014), pharyngitis (OR, 6.9; 95% CI, 1.5-30.2; p = 0.010), splenomegaly (OR, 5.4; 95% CI, 1.1-26.7; p = 0.039), and neutrophilic leukocytosis (OR, 18.1; 95% CI, 3.5-93.6; p = 0.001) were [ncbi.nlm.nih.gov]

  Pharyngitis Pharyngitis, non-suppurative occurs in approximately 70% of the patients [ 6 ]. Hepatomegaly with or without elevation in liver aminotransferases frequently occurs. [oapublishinglondon.com]

  2 Department of Internal Medicine, The University of Chicago Medical Center, Chicago, IL, USA Abstract: Adult-onset Still's disease (AOSD) – a multi-systemic inflammatory condition characterized by high fevers, polyarthritis, an evanescent rash, and pharyngitis [dovepress.com]

  Clinical features include quotidian fevers, arthralgias, arthritis, pharyngitis, lymphadenopathy and an evanescent rash. AOSD should be considered in the differential diagnosis of fever of unknown origin. [pagepress.org]

• Nausea

  Poor appetite, nausea, and weight loss may occur. Lymphadenopathy, splenomegaly, hepatomegaly, and sore throat are also common. Pericarditis, pleuritis, pericardial effusion and pleural effusion may occur but is less likely. [slideshare.net]

  Poor appetite, nausea, and weight loss are common. There is also commonly swelling of the lymph glands, enlargement of the spleen and liver, and sore throat. [rxlist.com]

• Hepatomegaly

  – Elevated acute-phase reactants: elevated ESR, CRP, white count, and hyperferritinemia (69-97% of patients) -Other findings that may be found include: myalgia, sore throat, hepatomegaly, splenomegaly, lymphadenopathy, serositis, abdominal pain, elevated [utswim.wordpress.com]

  Liver involvement was observed in all cases, with hepatomegaly in 81.3%, increased liver enzymes in 50.0%, and hypergammaglobulinemia in 68.8%. Cardiac involvement was observed in 12.6%, pleuritis in 6.3%, and renal involvement in 25.0%. [ncbi.nlm.nih.gov]

  Physical examination revealed pallor, hepatomegaly. [ijnm.in]

• Hepatosplenomegaly

  Adult-onset Still disease is an idiopathic systemic disorder that presents with relapsing high fever, joint pain, skin rash, pharyngitis, hepatosplenomegaly, and lymphadenopathy. [symptoma.com]

  Adult onset Still's disease (AOSD) is a systemic inflammatory disease characterized by high-fevers, articular involvement, maculopapular rash, hepatosplenomegaly, lymphadenopathy, and a neutrophilic leukocytosis. [ncbi.nlm.nih.gov]

  […] 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 [pathologyoutlines.com]

  HLH is also characterized by fever, hepatosplenomegaly, increased ferritin levels all of which are also features of AOSD. [ijpmonline.org]

  The presenting features are variable and may include high fever (>39°C) with daily spikes, sore throat or pharyngitis, arthralgia or arthritis (>65% of patients), transient maculopapular rash, and more rarely myalgia, lymphadenopathy, hepatosplenomegaly [orpha.net]

• Jaundice

  A 48-year-old woman was admitted because of spiking high fever, sore throat, and jaundice. [ncbi.nlm.nih.gov]

  Jaundice developed and the patient went into grade IV hepatic encephalopathy following which she died. [tropicalgastro.com]

  Look for signs of jaundice, pallor, purpuric rash, and encephalopathy in MAS. E. What diagnostic tests should be performed? There are a number of laboratory findings that are characteristic for adult JRA, however none are specific for the diagnosis. [clinicaladvisor.com]

• Arthritis

  Adult-onset Still's disease (AOSD) is a rare inflammatory disease characterized by the classical triad of daily fever, arthritis, and typical salmon-colored rash. [ncbi.nlm.nih.gov]

  Subtype-specific peripheral blood gene expression profiles in recent-onset juvenile idiopathic arthritis. Arthritis Rheum. 2009;60:2102–12. PubMedCentral PubMed CrossRef Google Scholar 41. [doi.org]

• Arthralgia

  PMN predominant. – Arthralgias: With or without synovitis. Occurs in 72-95% of patients. [utswim.wordpress.com]

  Arthralgia is usually correlated with fever spikes. [orpha.net]

  Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. [ncbi.nlm.nih.gov]

  AOSD, although uncommon, has a characteristic constellation of clinical and laboratory features and should be considered in the differential diagnosis of pyrexia of unknown origin associated with a rash and arthralgia. [e-ijd.org]

• Myalgia

  Five weeks earlier, sore throat, fever, malaise, and myalgias had developed. Broad-spectrum antibiotic therapy was administered, without improvement. A diagnosis was made. [blogs.nejm.org]

  Since March 2017, he has had progressive fatigue, myalgias, rash, weight loss, diarrhea, and recurrent low-grade fever. Subsequent bone marrow biopsy and FDG PET/CT demonstrated no active lymphoma. [ncbi.nlm.nih.gov]

  – Elevated acute-phase reactants: elevated ESR, CRP, white count, and hyperferritinemia (69-97% of patients) -Other findings that may be found include: myalgia, sore throat, hepatomegaly, splenomegaly, lymphadenopathy, serositis, abdominal pain, elevated [utswim.wordpress.com]

• Skin Rash

  Current diagnostic criteria are divided into major (Fever ≥ 39°C, joint pain, skin rash, pharyngitis, granulocytosis of ≥ 80%, and glycosylated ferritin fraction of ≤ 20%) and minor (leukocytosis of > 10,000/mm3 and a typical skin rash). [symptoma.com]

  Recently, a nonclassic, nonevanescent skin rash has been reported. We report a 27-year-old woman with AOSD showing persistent pruritic papular lesions. Histologically, dyskeratotic keratinocytes were seen in the upper epidermis. [ncbi.nlm.nih.gov]

  Skin rash develops in about of 95% of patients. [dermnetnz.org]

  Abstract Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology characterized by spiking fever, evanescent skin rash, arthralgia or arthritis, involvement of various organs, and predominantly neutrophilic leucocytosis [e-ijd.org]

• Eruptions

  Emerging data also suggest that patients with persistent eruption have a worse prognosis. [ncbi.nlm.nih.gov]

• Koebner Phenomenon

  It commonly appears on the trunk, neck, or extremities and may be associated with dermatographism, Koebner phenomenon (lesions arising at sites of trauma/pressure), pruritus, or urticaria. Fixed dermal plaques have been rarely described. [rheumaknowledgy.com]

  In one-third of patients, the rash is mildly pruritic and develops at sites of pressure or trauma (Koebner’s phenomenon). Arthralgia/Arthritis and Myalgia Intense arthralgias are a universal finding. [stillsdisease.org]

  Look for the rash in areas subject to pressure like the beltline and under the breasts or it can be elicited by stroking the skin (Koebner’s phenomenon). The rash is frequently misdiagnosed as a drug reaction. Figure 1. [clinicaladvisor.com]

  This type of eruption often had a linear configuration, possibly because of the Koebner phenomenon, thus resembling a flagellate erythema. [journals.lww.com]

  Cutaneous manifestations of AOSD: The common ones are Still’s Rash, Koebner phenomenon, Dermatographism. Uncommon: It includes Pruritis, Urticaria, Dermal plaques, Facial Rash, Alopecia, Erythema nodosumd and Raynaud’s phenomenon. [pjms.com.pk]

• Pruritus

  Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. [ncbi.nlm.nih.gov]

  It commonly appears on the trunk, neck, or extremities and may be associated with dermatographism, Koebner phenomenon (lesions arising at sites of trauma/pressure), pruritus, or urticaria. Fixed dermal plaques have been rarely described. [rheumaknowledgy.com]

• Eczema

  Allergic Eczema... Academy of Nutrition and Dietetics The food you eat determines the quality of your life. It affects your appearance, may prevent some diseases... [doi.org]

• Renal Insufficiency

  Renal Insufficiency. Avoid NSAIDS in patients with significant renal impairment. Doses of methotrexate may need be reduced by 50% in patients with creatinine clearance (CrCl) < 50 milliliters/minute (mL/min). [clinicaladvisor.com]

  HIVAN was characterized by a black ethnic predominance, massive proteinuria, rapidly progressive renal insufficiency and distinct pathological findings. [rheumatology.oxfordjournals.org]

• Kidney Failure

  Specifically, 10 patients died of uncontrollable MAS, two of severe kidney failure requiring dialysis, two of multiple organ failure, and two of severe infection related to the immunosuppressive therapy. [bmcmedicine.biomedcentral.com]

The diagnosis of AOSD is primarily a one of exclusion, as numerous autoimmune, malignant, and infectious diseases induce the nonspecific signs and symptoms seen in this condition [1] [3] [7] [8]. Thus, a comprehensive clinical and laboratory workup is necessary. Firstly, during a history taking, patients should be asked about the course of the disease and the appearance of symptoms, whereas a thorough physical examination has an important role in identifying lymphadenopathy, cutaneous changes, joint inflammation, and hepatosplenomegaly.

A complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serum ferritin should be evaluated in laboratory studies [1] [3] [4] [7] [8]. Additionally, rheumatoid factor (RF) and antinuclear antibodies (ANA), both performed to exclude conditions such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), are also recommended in the initial workup [3].

Current diagnostic criteria are divided into major (Fever ≥ 39°C, joint pain, skin rash, pharyngitis, granulocytosis of ≥ 80%, and glycosylated ferritin fraction of ≤ 20%) and minor (leukocytosis of > 10,000/mm3 and a typical skin rash) [3] [4] [7] [8]. AOSD is confirmed if ≥ 4 major criteria or 3 major and 2 minor criteria are met [1] [3] [4] [7].

• Enlargement of the Liver

  Its symptoms usually include persistent fever, fugitive salmon-colored rash, arthritis, sore throat (not specific), but it may also lead to internal organs' involvement, which presents with enlargement of the liver and spleen, swollen lymph nodes, carditis [ncbi.nlm.nih.gov]

  The disease typically presents with joint pain, high fevers, a salmon-pink macular or maculopapular rash, enlargement of the liver and spleen, swollen lymph nodes, and a neutrophil-predominant increased white blood cell count in the blood. [en.wikipedia.org]

• Thrombocytosis

  Both patients had marked leukocytosis with neutrophil predominance, thrombocytosis, elevated liver enzymes and elevated acute phase rectants. [ajol.info]

  A peripheral smear also showed neutrophilia and thrombocytosis. [ijdvl.com]

  Blood cell count for improvement in leukocytosis and thrombocytosis. [clinicaladvisor.com]

  Investigations revealed hemoglobin of 9.7 g/dL, polymorphonuclear leukocytosis total leukocyte count (TLC 17500/mm 3 with 92% neutrophils), thrombocytosis (platelet count 848000/mm 3 ), a markedly raised erythrocyte sedimentation rate (ESR) (45 mm in [ijnm.in]

  Neutrophilic leukocytosis (WBC > 12.5), thrombocytosis, markedly elevated ESR (50% are >90 mm/hr and 90% are > 50 mm/hr) or C-reactive protein levels (> 2.0 mg/dl). [rheumaknowledgy.com]

• Neutrophilia

  Adult Still’s Disease (ASD) is a systemic inflammatory disorder of unknown etiology, typically characterized by a clinical triad (daily spiking high fevers, evanescent rash, arthritis), and a biological triad (hyperferritinemia, hyperleukocytosis with neutrophilia [profiles.uonbi.ac.ke]

  Keywords Meningitis Adult onset Still’s disease Neutrophilia This is a preview of subscription content, log in to check access. Notes Conflict of interest statement The authors declare that there is no conflict of interest. References 1. [doi.org]

  A peripheral smear also showed neutrophilia and thrombocytosis. [ijdvl.com]

  Accordingly, the sample preferably originates from a patient who has been diagnosed with having or diagnosed with being free from at least one of the parameters selected from the group of arthritis, fever, leukocytosis, neutrophilia, an eleveated serum [google.st]

  Leucocytosis is the result of a striking neutrophilia that is probably secondary to bone marrow granulocyte hyperplasia (Pouchot et al. 1991 and Min et al. 2003 ). [springerplus.springeropen.com]

Treatment failed in four cases (two with each anti-TNF-α) [ 15 ]. [doi.org]

We report the successful use of abatacept in the treatment of a patient with AOSD manifested by polyarthritis, rash, fevers, elevated liver function tests, and ferritin levels refractory to treatment with methotrexate and hydroxychloroquine. [ncbi.nlm.nih.gov]

Management and treatment The aim is to achieve complete remission and prevent joint damage through treatment. Treatment-free remission is possible but the risk of relapse remains present throughout life. [orpha.net]

Prognosis The overall prognosis is generally good, once acute life-threatening manifestations have been controlled. Some patients with chronic disease and major joint involvement may have significantly altered quality of life. [orpha.net]

Systemic manifestations, per se, did not contribute to poor functional prognosis. Chronic articular disease had the worst outcome: 27% evolved to functional class III status, compared with none in the cyclic systemic groups. [ncbi.nlm.nih.gov]

What is the Prognosis of Adult-Onset Still’s Disease? (Outcomes/Resolutions) The prognosis of Adult-Onset Still’s Disease may be assessed on a case-by-case basis. [dovemed.com]

The disease is an entity with heterogeneous pathology; and diverse suggested etiologies, clinical manifestations and prognoses. [ncbi.nlm.nih.gov]

Etiology The etiology of AOSD and its underlying pathogenetic mechanisms are not known. No risk factors for the disease have been identified so far, but environmental factors are suspected. [orpha.net]

Our study aims to analyze different aspects of articular manifestations complicating adult onset Still disease to define epidemiological, clinical and evolving characteristics of these complications. [panafrican-med-journal.com]

Summary Epidemiology Prevalence and incidence data are difficult to determine given the broad, non-specific clinical presentation. The estimated prevalence of AOSD is more than 1/100,000 population. [orpha.net]

[…] 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 Epidemiology [pathologyoutlines.com]

Magadur-Joly G et al (1995) Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 54(7):587–590 PubMed CrossRef Google Scholar 4. [doi.org]

References Magadur-Joly G, Billaud E, Barrier JH, Pennec YL, Masson C, et al. (1995) Epidemiology of adult Still's disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 54: 587. [archivesofmedicine.com]

Recent accumulation of knowledge, mostly arising from hereditary autoinflammatory diseases and from the systemic-onset juvenile idiopathic arthritis (sJIA), has given raise to new hypotheses on the pathophysiology of AOSD. [ncbi.nlm.nih.gov]

More important, novel biologic therapies have offered important clues to AOSD's underlying pathophysiology. [dovepress.com]

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 [pathologyoutlines.com]

Additional research into the pathophysiology of PNGD is also warranted, as this would optimize medical management. [archivesofmedicine.com]

Autoinflammatory syndromes and cellular responses to stress: pathophysiology, diagnosis and new treatment perspectives. Best Pract Res Clin Rheumatol. 2012;26:505–33. PubMed CrossRef Google Scholar 52. [doi.org]

Clinicians need to be aware of this condition so that they can correctly diagnose it and prevent its serious complications. [ncbi.nlm.nih.gov]

It is important to keep the inflammation under control to prevent more damage to the body. If prednisone is taken for a long time, a doctor may recommend taking calcium and vitamin D to prevent thinning of the bones. [arthritis.org]

When steroids are used they will often be given with other drugs to help to prevent side-effects. [nras.org.uk]

REFERENCE BOOK Screening for Colorectal Cancer | CTFPHC REFERENCE BOOK Screening for Cognitive Impairment | CTFPHC REFERENCE BOOK Screening for Depression | CTFPHC REFERENCE BOOK Screening for Developmental Delay | CTFPHC Review Canadian Task Force on Preventive [qxmd.com]

Treatment aims to control inflammation, and prevent joint damage and other complications. [rarediseases.info.nih.gov]

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