Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 6  |  Issue : 4  |  Page : 465-467  

Adult onset Still's disease: An uncommon etiology of non-resolving pneumonia


1 Department of Medicine, College of Medicine & Jawaharlal Nehru Memorial Hospital, West Bengal University of Health Sciences, Kalyani, Nadia, West Bengal, India
2 Department of ENT, PGIMSR & ESI Hospital, Joka, Kolkata, India
3 Department of Neuromedicine, N. R. S. Medical College, Kolkata, West Bengal, India

Date of Web Publication17-Sep-2013

Correspondence Address:
Somak Kumar Das
A / 14, Katjunagar, Jadavpur, Kolkata - 700 032
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.118293

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  Abstract 

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology and pathogenesis, usually presenting with high spiking fever accompanied by several systemic manifestations. Pulmonary complications in AOSD are also rare. Only a few earlier pathologic reports exist describing AOSD with pulmonary involvement. AOSD presenting as non-resolving pneumonia is poorly reported in literature. We report one case of AOSD presented initially as non-resolving pneumonia. In this report, we conclude that every clinician must consider AOSD as a potential differential diagnosis of non-resolving pneumonia.

Keywords: Adult onset Still′s disease, hyperferritinemia, non-resolving pneumonia


How to cite this article:
Das SK, Nath T, Ghosal A, Kundu AK. Adult onset Still's disease: An uncommon etiology of non-resolving pneumonia. Med J DY Patil Univ 2013;6:465-7

How to cite this URL:
Das SK, Nath T, Ghosal A, Kundu AK. Adult onset Still's disease: An uncommon etiology of non-resolving pneumonia. Med J DY Patil Univ [serial online] 2013 [cited 2022 Aug 17];6:465-7. Available from: https://www.mjdrdypu.org/text.asp?2013/6/4/465/118293


  Introduction Top


Adult-onset Still's disease (AOSD) is a rare inflammatory disorder of unknown etiology. Its main features are high spiking fever, evanescent salmon colored rash, polyarthralgia, lymphadenopathy, hepatosplenomegaly, leukocytosis with neutrophilic predominence, and abnormal liver enzymes, elevated erythrocyte sedimentation rate (ESR) and serum ferritin. Pulmonary manifestations of AOSD are rare. Pulmonary manifestations described earlier are pleuritis and transient radiologic infiltrations. [1] A few reports exist in literature describing AOSD presenting initially as non-resolving pneumonia. [2] We report a case of AOSD presented initially as non-resolving pneumonia and treated successfully with immunosuppressive agents. We conclude that every clinician must consider AOSD as an important differential diagnosis of non-resolving pneumonia.


  Case Report Top


A 24-year-old lady presented with high grade spiking fever (39-39.6°C), productive cough, dyspnea and pain on right side of the chest for 4 weeks prior admission. Within a span of 4 weeks, she consulted a general physician and a pulmonologist. Records of the physical examinations revealed mild anemia, absence of jaundice, clubbing, and lymphadenopathy, and presence of bronchial breath sound at right lower and middle lobe on chest auscultation. Physical examinations did not reveal any organomegaly at that time. Chest X-ray [Figure 1] showed radio-opaque shadows at right lower and middle zone indicating parenchymal infiltrations and pleural effusion.
Figure 1: Chest X-ray posterior anterior view showing moderate pleural effusion and infiltrations in right middle and lower zones

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At our center, we performed a computed tomography of the thorax [Figure 2], which confirmed right lower and middle lobe consolidation, and moderate pleural effusion. Hematological investigations showed leucocytosis of 33.7 × 10 9 /L with 90% neutrophils. She was empirically treated with multiple antibiotics in several successions for the next 4 weeks without any improvement. However, with the advancement of time, she developed polyarthritis involving proximal interphalangeal, metacarpophalangeal, wrists, and knee joints of both sides. We noticed that the polyarthritis was so disabling, she could hardly ambulate on her own. Time to time evanescent salmon colored rashes developed mainly over her trunk. Meticulous physical examination revealed newly developed hepatosplenomegaly. Hematological investigations revealed anemia and persistent leucocytosis of 18.4 × 10 9 /L with more than 83% neutrophils. The ESR and acute phase reactant like C-reactive protein was very high; the ESR was 97 mm/1 st h (Westergren) with C-reactive protein 156.4 mg/L. There were markedly elevated levels of serum ferritin (33,593 μg/L). Anti-cyclic citrullinated peptide, antinuclear antibody (ANA) and rheumatoid factor (RF) were negative. Liver enzymes (alanine transaminase 207 U/L, and aspartate transaminase 162 U/L) were elevated. Renal and coagulation profiles were within normal limits. Gram staining and Ziehl-Neelsen acid-fast staining of sputum did not show any causative organisms. Blood, sputum, and urine cultures also revealed no evidence of bacterial, fungal or viral infection.
Figure 2: Computed tomography of thorax showing consolidation and pleural effusion on right lower lobe

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Based on her clinical features and review of the laboratory parameters, she was diagnosed to have AOSD using the Yamaguchi criteria, and was started on naproxen 250 mg twice daily, hydroxychloroquine 400 mg daily and prednisolone 40 mg once daily. Over the next few days, the patient became afebrile; all her respiratory problems were diminished in intensity. Repeated hematological investigations over next 2 weeks showed gradual decrease in total leucocyte counts to 7.4 × 10 9 /L with 65% neutrophils. Repeat chest X-ray showed resolution of pneumonia as well as pleural effusion. The arthralgia and arthritis improved, and she became ambulant after 2 weeks. The ESR and acute phase reactant like C-reactive protein gradually diminished; the ESR was 29 mm/1 st h (Westergren) with C-reactive protein 3.7 mg/L after 2 weeks' of prednisolone therapy. The patient was discharged after 4 weeks on prednisolone 30 mg daily with a tapering dose of 5 mg weekly and hydroxychloroquine 400 mg daily. As AOSD generally requires long-term management, she was advised to come in next 2 weeks for follow-up.


  Discussion Top


AOSD is an uncommon immunological disorder of unknown etiology with recognized increased frequency in adults. AOSD was first described by Eric Bywaters in 1971. [3] The prevalence of AOSD is estimated to be 1/100,000 people. The diagnosis of AOSD is solely clinical and is a "diagnosis of exclusion" as serological markers and specific diagnostic tests do not exist, although a very high serum ferritin level does support the diagnosis. Levels in serum of 30,000 μg/L have been reported in some patients with highly active disease; when levels are greater than 10,000 μg/L, physicians should strongly consider AOSD as the diagnosis. [4] The Yamaguchi criteria (1992), is the most widely used criteria to diagnose AOSD with 93.5% sensitivity. [5] Five or more criteria must be met in order to make a diagnosis of AOSD, including 2 or more major criteria, after excluding infections, malignancies or rheumatic diseases. Macrophage activation syndrome must be kept in mind as a diagnostic possibility in patients having high serum ferritin level.

The frequency of pulmonary manifestations in AOSD is 12-53% for pleuritis and 0-27% for interstitial pneumonia. [5],[6] Pleuritis is often seen soon after onset of AOSD or during acute exacerbation of AOSD. Interstitial pneumonia is acute and it is detected as a transient infiltrative shadow. Recently, Hijikata et al. reported a case of AOSD in which transbronchial lung biopsy (TBLB) revealed a pattern of organizing pneumonia. [2] However, cases of AOSD presenting pulmonary manifestation detected by TBLB or bronchoalveolar lavage are quite rare. [2] AOSD generally requires long term management. Oral glucocorticoids are often needed to control systemic symptoms. It is reasonable to prescribe weekly methotrexate to help control the inflammation and serve as a steroid-sparing drug. Because of the high likelihood that this is an example of a cytokine-driven disease, use of Tumor necrosis factor - alpha or Interleukin-6 blockers might be effective.

Non-resolving pneumonia occurs as a result of inappropriate antimicrobial therapy, superinfection, inadequate host response, obstruction, empyema, non-infectious processes, or recurrent infection. Possibilities of non-infectious pathology should considered if Gram stained sputum culture is unrevealing, response to antimicrobial therapy proves unsatisfactory, or radiologic findings are atypical. The presence of rashes, lymphadenopathy, splenomegaly, and arthralgia favor rheumatic origin of pulmonary involvement.

In our patient the diagnosis of AOSD was made based on the Yamaguchi criteria following clinical features : s0 piking fever, rash, arthralgia, splenomegaly, leukocytosis, elevated levels of serum aminotransferase, absence of RF and ANA, and a strikingly high serum ferritin level. We strongly believe lung consolidation in this patient was due to AOSD as intensive investigations failed to found any infectious, malignant, and common rheumatic pathology. Moreover, the patient did not even respond to extensive antimicrobial therapy, but promptly responded to oral corticosteroid therapy within 2-3 weeks, indicating some immunological aberration underneath. So, we conclude that every clinician should consider AOSD as an important differential diagnosis of non-resolving pneumonia, and should be aware that non-resolving pneumonia can be a complication of AOSD in some cases.

 
  References Top

1.Cheema GS, Quismorio FP Jr. Pulmonary involvement in adult-onset Still's disease. Curr Opin Pulm Med 1999;5:305-9.  Back to cited text no. 1
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2.Hijikata N, Takayanagi N, Sugita Y, Kawabata Y. Adult-onset Still's disease with pulmonary involvement. J Bronchology Interv Pulmonol 2009;16:277-82.  Back to cited text no. 2
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3.Bywaters EG. Still's disease in the adult. Ann Rheum Dis 1971;30:121-33.  Back to cited text no. 3
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4.Van Reeth C, Le Moel G, Lasne Y, Revenant MC, Agneray J, Kahn MF, et al. Serum ferritin and isoferritins are tools for diagnosis of active adult Still's disease. J Rheumatol 1994;21:890-5.  Back to cited text no. 4
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5.Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992;19:424-30.  Back to cited text no. 5
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6.Ohta A, Yamaguchi M, Tsunematsu T, Kasukawa R, Mizushima H, Kashiwagi H, et al. Adult Still's disease: A multicenter survey of Japanese patients. J Rheumatol 1990;17:1058-63.  Back to cited text no. 6
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