J Korean Med Sci.  2009 Feb;24(1):155-157. 10.3346/jkms.2009.24.1.155.

A Case of Adult-Onset Still's Disease Complicated with Diffuse Alveolar Hemorrhage

• Sari I
• Birlik M
• Binicier O
• Akar S
• Yilmaz E
• Onen F
• Akkoc N

• KMID: 1794423
• DOI: http://doi.org/10.3346/jkms.2009.24.1.155

Abstract

Adult-onset Still's disease (AOSD) is an inflammatory disease that presents with a variety of clinical symptoms. Pulmonary involvement is well-known in AOSD and is seen in up to 53% of AOSD cases, with the most common pulmonary diseases being pleural effusion and transient pulmonary infiltrates. We present the first case of chronic AOSD complicated with diffuse alveolar hemorrhage during the acute flare of the disease.

Keyword

Still's Disease, Adult-Onset; Lung Diseases

MeSH Terms

Adult
Ankylosis/diagnosis
Anti-Inflammatory Agents/administration & dosage
Diagnosis, Differential
Female
Fever of Unknown Origin/diagnosis
Hemorrhage/*diagnosis/drug therapy/etiology
Humans
Lung Diseases/*diagnosis/drug therapy/etiology
Methylprednisolone/administration & dosage
*Pulmonary Alveoli
Still's Disease, Adult-Onset/complications/*diagnosis/drug therapy/radiography
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 This radiography image of the patient's hands shows bilateral ankylosis of the carpal joints. Note the narrowing and cystic changes on the second metacarpophalangeal joints.

• 

  Fig. 2 (A) A chest radiograph shows bilateral consolidation in the middle and lower lung zones. Blunting of the costophrenic angle is seen in the right lung. (B) A CT image shows extensive areas of ground-glass attenuation and airspace consolidation due to alveolar hemorrhage in both lungs. The bilateral pleural effusions are also notable.

• 

  Fig. 3 Ten days after pulsed methylprednisolone treatment, the chest radiograph (A) and CT image (B) obtained at the subcarinal level were normal.

Reference

1. Cush JJ. Adult-onset Still's disease. Bull Rheum Dis. 2000. 49:1–4.

2. Kadar J, Petrovicz E. Adult-onset Still's disease. Best Pract Res Clin Rheumatol. 2004. 18:663–676.

3. Nishimagi E, Hirata S, Kawaguchi Y, Okamoto H, Hara M, Kamatani N. Myocardial dysfunction in a patient with adult-onset Still's disease (AOSD). Clin Exp Rheumatol. 2004. 22:506–507.

4. Garrote FJ, Marco J, Obeso G, Rodriguez E, del Ser T. Aseptic meningitis and focal central nervous system involvement in a case of adult onset Still's disease. J Rheumatol. 1993. 20:765–767.

5. Bennett AN, Peterson P, Sangle S, Hangartner R, Abbs IC, Hughes GR, D'Cruz DP. Adult onset Still's disease and collapsing glomerulopathy: successful treatment with intravenous immunoglobulins and mycophenolate mofetil. Rheumatology (Oxford). 2004. 43:795–799.

6. Kuo HL, Huang DF, Lee AF. Thrombotic microangiopathy in a patient with adult onset Still's disease. J Clin Rheumatol. 2002. 8:276–280.

7. Cheema GS, Quismorio FP Jr. Pulmonary involvement in adult-onset Still's disease. Curr Opin Pulm Med. 1999. 5:305–309.

8. Collard HR, Schwarz MI. Diffuse alveolar hemorrhage. Clin Chest Med. 2004. 25:583–592.

9. Schwarz MI, Fontenot AP. Drug-induced diffuse alveolar hemorrhage syndromes and vasculitis. Clin Chest Med. 2004. 25:133–140.

10. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992. 19:424–430.

11. Efthimiou P, Georgy S. Pathogenesis and management of adult-onset Still's disease. Semin Arthritis Rheum. 2006. 36:144–152.

12. Arndt PG, Fantuzzi G, Abraham E. Expression of interleukin-18 in the lung after endotoxemia or hemorrhage-induced acute lung injury. Am J Respir Cell Mol Biol. 2000. 22:708–713.

13. Yang YJ, Chen SH, Ge XR. Role of interleukin-18 in the development of acute pulmonary injury induced by intestinal ischemia/reperfusion and its possible mechanism. J Gastroenterol Hepatol. 2007. 22:253–260.