Still's disease

Introduction: Adult onset Still's disease (AOSD) is a disorder characterized by fever, rash, and arthritis. Multi-organ involvement has occasionally been reported, however adult respiratory distress syndrome (ARDS) is quite rare due to AOSD. We report the case of a patient with ARDS due to AOSD.

Case presentation: A 22 year old previously healthy male presented with complaints of fever, sore throat, diffuse arthralgias, myalgias, and chest pain. The fevers had been present for 6 weeks, and a fleeting cutaneous rash had been noted prior to admission. Shortness of breath and hypoxemia developed, and the patient was transferred to the intensive care unit. Numerous antibiotics had been given without resolution of the fever, including erythromycin, doxycycline, and clindamycin. Past medical history was significant only for an episode of arthritis of the right knee 5 years previously. There was no history of IV drug use, HIV, recent TB exposure, or exposure to farm or exotic animals. There was a history of travel to Japan and southeast Asia in the past year. Current medications included vancomycin, levofloxacin, and acetaminophen. Physical exam: temperature 102.2, heart rate 115, blood pressure 140/80, respiratory rate 30. The patient appeared toxic in general. The oropharynx was unremarkable, and there was no significant adenopathy. The lungs revealed rales bilaterally, with decreased breath sounds in the bases. The heart sounds revealed no murmur or rub. The abdomen was soft, and the spleen was palpable. The extremities revealed a moderate amount of peripheral edema, and there was an erythematous maculopapular rash on the arms and trunk. The neurological exam was non-focal. Laboratory: hemoglobin 9.0 G/dl, white blood count 10.8 K/UL with 80% neutrophils, platelet count 195 K/UL; renal function was normal, liver enzymes were elevated: Aspartate aminotransferase (AST) 8.54 U/L, alkaline phosphatase 497 U/L, lactate dehydrogenase 2389 U/L. Radiology: chest radiograph showed diffuse, bilateral pulmonary infiltrates; chest and abdomen computed tomogram showed no adenopathy, moderate sized pleural effusions, and moderate hepato-splenomegaly. A trans-thoracic echocardiogram revealed a small pericardial effusion. Hospital course: Intubation and mechanical ventilation with positive end expiratory pressure were required for progressive respiratory failure. Multiple serologies were negative, including HIV, hepatitis A, B, and C, lyme, rickettsia, ebstein-barr virus (EBV), and cytomegalovirus. Brucella was positive by latex agglutination at 1:640, however was negative by enzyme linked immuno-assay. Tests for anti-nuclear antibody were negative. Bronchoscopy was performed which revealed no alveolar hemorrhage. Microbiologic studies on bronchoalveolar lavage were negative, including tests for fungus, acid fast bacilli, pneumocystis, legionella, cytomegalovirus, herpes virus, and bacteria. A skin biopsy revealed nonspecific inflammatory changes with no evidence of a leukocytoclastic vasculitis. Serum ferritin was ordered. A diagnosis of adult onset still's disease was considered, and treatment with high dose pulse intravenous methylprednisolone was given. A dramatic response was seen, with resolution of fever, improvement in oxygenation, a decrease in the liver enzymes, and improvement of the rash. The ferritin returned at 83,100 ng/ml. The patient was extubated 3 days following initiation of corticosteroids, with continued clinical improvement.

Discussion: AOSD can present with multi organ involvement, however there have been only a few eases of acute respiratory failure due to AOSD. Clinical manifestations can include sore throat, mental status changes, myositis, pericarditis, lymphadenopathy, renal and liver involvement, anemia, thrombocytopenia, and coagulopathy. pulmonary manifestations include an exudative pleural effusion in up to 50% of patients, pulmonary infiltrates in 10 to 30%, interstitial pneumonitis in up to 6%, and rare cases of ARDS. Open lung biopsy in patients with pneumonitis has revealed nonspecific inflammation and patchy areas of interstitial fibrosis. Major criteria for the diagnosis of AOSD include fever greater than 2 weeks, a cutaneous rash, polyarthritis, leukocytosis, or history of childhood Still's disease; minor criteria include arthralgias, myalgias, pericarditis, increased SGOT, and pharyngitis. Diagnosis is made with 4 major criteria or 3 major and 3 minor criteria. Exclusionary criteria include the presence of infection, malignancy or a connective tissue disease. The pathophysiology is unknown, however production of proinflammatory cytokines may be important. Hyperferritinemia is a non-specific but often elevated finding; ferritin levels greater than 10,000 ng/ml have been reported in patients with AOSD. Treatment of AOSD with systemic involvement is with corticosteroids. High dose pulse methylprednisolone is recommended for fulminant disease. Prognosis is generally favorable, including patients who manifest with ARDS and are treated with corticosteroids. There are 4 reports of ARDS due to AOSD in the English literature; 3 were treated successfully with corticosteroids.

Conclusion: ARDS is a rare manifestation of AOSD. AOSD should be considered in the differential diagnosis of patients presenting with fever and rash, particularly if they also have arthritis and leukocytosis. Hyperferritenemia may be present, and treatment with corticosteroids may be life saving.

David R. Clark, MD, T. Dwyer, K. O'Neil, FCCP--National Naval Medical Center, Bethesda, MD

COPYRIGHT 1999 American College of Chest Physicians
COPYRIGHT 2000 Gale Group