Symptoms, Symptoms Everywhere, but What Diagnosis to Make? Systemic‐Onset Juvenile Idiopathic Arthritis

1Georgetown School of Medicine, Washington, DC
2Georgetown University Hospital, Washington, DC

Meeting: Hospital Medicine 2014, March 24-27, Las Vegas, Nev.

Abstract number: 264

Case Presentation:

A 4 year‐old boy presented with fever, generalized joint pain, and scrotal edema. He reported emesis, chest pain, dyspnea, and non‐bloody diarrhea. Initial exam noted diffuse pulmonary crackles and a macular rash. He later developed café‐a‐lait spots and beefy red tonsils with a white exudate. There was no active synovitis. Initial labs showed anemia, leukocytosis, and elevated ESR, CRP and LDH. Further tests, including urinanalysis, coagulation studies, and quantitative immunoglobulins, were normal. Rheumatoid factor and autoimmune antibodies were negative. Echocardiogram revealed RV dilatation with abnormal interventricular septal motion and trivial pericardial effusion. Joint evaluations by radiography were normal. CT and ultrasound noted mild abdominal lymphadenopathy and hepatomegaly without testicular torsion. Extensive evaluations for bacterial, viral and atypical infections were negative. Bone marrow biopsy and chromosomal analysis were normal. Based on his symptoms and pertinent negative findings, he was diagnosed with systemic‐onset juvenile idiopathic arthritis (SOJIA). He was started on furosemide, acetaminophen, and ibuprofen for symptom relief and then initiated on prednisolone with subsequent improvement.

Discussion:

Pediatric hospitalists often encounter serious systemic illnesses that present with non‐specific symptomatology. A broad differential coupled with a meticulous history and judicious diagnostic testing is essential for the correct diagnosis. SOJIA is a prime example of the need for such a rigorous workup. SOJIA commonly presents with only fever and polyarticular joint pain, but can be accompanied by rash, lymphadenopathy, or hepatosplenomegaly. However, these symptoms are not unique to SOJIA and may manifest in other autoimmune diseases, hemophagocytic lymphohistiocytosis, malignancy, and even viral or atypical infections. Frustratingly, when diagnosis is delayed, SOJIA can progress to uveitis, myopericarditis, and congestive heart failure. Lab markers for SOJIA are non‐specific. Unlike other juvenile arthritis subtypes, rheumatoid factor and ANA are negative in SOJIA. Due to SOJIA’s vague presentation and variable symptoms, it is considered a diagnosis of exclusion. Even when autoimmune disease is correctly suspected, SOJIA is commonly misdiagnosed as incomplete Kawasaki disease due to the presence of coronary artery dilatation. Erroneously initiating IVIG for incomplete Kawasaki in patients who have SOJIA will not improve the disease, but can lead to life‐threatening macrophage activation syndrome. After a correct diagnosis, SOJIA patients can be treated successfully with steroids, NSAIDS, and DMARDs.

Conclusions:

Systemic‐onset idiopathic juvenile arthritis (SOJIA) is a diagnosis of exclusion that can present with a broad constellation of symptoms and mimic many other autoimmune, hematological, and infectious diseases. In evaluating patients with non‐specific inflammatory symptoms, hospitalists should consider SOJIA and perform an appropriate workup to avoid a misdiagnosis.

To cite this abstract:

Eng V, Gandiga P. Symptoms, Symptoms Everywhere, but What Diagnosis to Make? Systemic‐Onset Juvenile Idiopathic Arthritis. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 264. Journal of Hospital Medicine. 2014; 9 (suppl 2). https://www.shmabstracts.com/abstract/symptoms-symptoms-everywhere-but-what-diagnosis-to-make-systemiconset-juvenile-idiopathic-arthritis/. Accessed March 28, 2020.

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