Downloaded from http://pmj.bmj.com/ on April 7, 2015 - Published by group.bmj.

com

97

CASE REPORT

Adult Still’s disease and respiratory failure in a 74 year

old woman
G S Stoica, R I Cohen, L J Rossoff
.............................................................................................................................

Postgrad Med J 2002;78:97–98

A case of adult onset Still’s disease in an elderly woman,

that was associated with severe respiratory failure and
multiorgan dysfunction, is reported. Histopathology was
confirmed on open lung biopsy.

A
dult onset Still’s disease is an uncommon rheumatologi-
cal syndrome, usually encountered in persons 16–35
years of age, with only one previous case reported in the
elderly.1 Pulmonary involvement is unusual and severe
respiratory failure and biopsy confirmation rare.2–8

CASE REPORT
A 74 year old woman with mild controlled hypertension pre-
sented with two months’ complaint of migratory, bilateral Figure 1 Portable anteroposterior chest radiograph with bilateral
interstitial infiltrates and effusions.
shoulder and knee arthralgias, myalgias, and intermittent
high fevers. This followed an initial three day episode of sore
throat treated unsuccessfully with several courses of antibiot-
ics and non-steroidal anti-inflammatory drugs (NSAIDs). The
persistent fever, rising leucocyte count, and anaemia
prompted admission to hospital. On physical examination she
was thin and in mild respiratory distress. Her temperature was
38.7°C, pulse 130 beats/min and regular, respiratory rate 20
breaths/min, and blood pressure 128/76 mm Hg. Heart and
lung examinations were normal. Severe pain was elicited on
palpation of shoulders and knees with a decreased range of
movement. There was no evidence of a rash, change in skin
turgor, active arthritis, or joint swelling. The rest of the exam-
ination was unremarkable.
Laboratory findings included a leucocyte count of 20 × 109/l,
with 86% neutrophils, and a haemoglobin concentration of 69
g/l (mean corpuscular volume 74 fl, serum ferritin 892 µg/l).
Total iron binding capacity and haptoglobin were normal.
Reticulocyte count was 2.2%, platelet count 275 × 109/l, and
Coombs test was negative. Electrolytes and renal and liver
function tests were all normal. The sedimentation rate was 85
mm/hour with antinuclear antibody, rheumatoid factor, Figure 2 Wedge resection of left lower lobe (hematoxylin and
antineutrophil cytoplasmic antibody, and anti-dsDNA all eosin stain, original × 100). There is evidence of extensive diffuse
negative. Serum protein electrophoresis, complement C3 and interstitial fibrosis, hyperplasia of type II pneumocytes, fibroblast
C4, urine analysis, blood cultures, and the electrocardiogram proliferation, and thickened alveolar septa.
were all normal. The chest radiograph revealed no infiltrates
with borderline cardiomegaly.
Initial treatment included empiric vancomycin, gentamy- the chest demonstrated non-specific pulmonary fibrosis.
cin, and ciprofloxacin for presumed infection (penicillin Transbronchial biopsies via fibreoptic bronchoscopy revealed
allergy) pending cultures. Her purified protein derivative was acute inflammation without granulomatous disease and all
negative but she was anergic. A bone marrow biopsy was cultures were negative. Ceftazidime and tobramycin were
normal. substituted in response to continuing fevers and leucocytosis.
On the fourth evening she developed a temperature of A thoracentesis revealed an exudate with negative Gram stain,
38.9°C and a transient maculopapular, pruritic, pink rash. Over cultures, and cytology. Her respiratory distress worsened and
the next few days she became progressively dyspnoeic with a she required intubation and mechanical ventilation. A
room air arterial blood gas revealing a pH of 7.49, carbon
dioxide pressure of 3.73 kPa, and oxygen pressure of 526.93
.............................................................
kPa. A repeat radiograph (fig 1) showed diffuse bilateral infil-
trates and small pleural effusions. Computed tomography of Abbreviations: NSAIDs, non-steroidal anti-inflammatory drugs

www.postgradmedj.com
 Downloaded from http://pmj.bmj.com/ on April 7, 2015 - Published by group.bmj.com

98 Stoica, Cohen, Rossoff

diagnosis of adult Still’s disease was entertained, antibiotics Two of the only three reports of histopathologicaal lung
discontinued, and a course of intravenous pulse steroids (1 g involvement in adult onset Still’s disease were based on trans-
of methylprednisolone per day for three days) started, bronchial biopsy and one at necropsy.3 7 8 Ours is the only
followed by a corticosteroid taper. The absence of significant reported open lung biopsy. Findings include non-specific
clinical improvement prompted a five day course of intra- fibrosis, thickened alveolar walls, hyperplasia of type II pneu-
venous gammaglobulin and open lung left lower lobe wedge mocytes, hyaline membrane formation, and lymphocytic
biopsy. Histology (fig 2) revealed extensive diffuse interstitial infiltration. In our case a large wedge resection (8 × 2.8 × 0.9
fibrosis with organising pneumonitis but no evidence of cm) revealed uniform features throughout (fig 2). There was
infection. She improved clinically, became afebrile, and was extensive diffuse interstitial fibrosis with some hyperplasia of
successfully extubated. Within a week, on corticosteroids, her type II pneumocytes. Additionally, focal infiltration with neu-
respiratory status deteriorated. She was reintubated, devel- trophils and foamy histiocytes was evident within the alveolar
oped an autoimmune and alloimmune thrombocytopenia lumina. There was no evidence of vasculitis or granulomatous
with a severe bleeding diathesis, and died within two weeks. inflammation.
The disease can have an acute or chronic form. The acute
DISCUSSION form usually responds to NSAIDs alone in about 25% of
patients.9 Patients who fail to respond to NSAIDs, those with
Adult onset Still’s disease is identical to that described in chil-
severe disease, coagulopathy, or increased liver function tests
dren with systemic juvenile rheumatoid arthritis. In children
require systemic corticosteroids, usually in the form of
systemic manifestations are usually self limited and rarely life
prednisone 0.5–1 mg/kg/day. Pulse corticosteroids (for exam-
threatening. The aetiology is unknown and it may complicate ple, methylprednisolone), intravenous gammaglobulin, im-
viral or other infection, with inconclusive linkage to HLA anti- munosuppressive, and/or cytotoxic agents appear indicated in
gens and stress.2 9 life threatening acute disease.2
There are no specific laboratory tests and pathognomonic
rash, described by Still, is transient and easily missed. The dif- .....................
ferential diagnosis includes vasculitides (for example, polyar- Authors’ affiliations
teritis nodosa, Wegener’s granulomatosis), connective tissue G S Stoica, R I Cohen, L J Rossoff, Long Island Jewish Medical Center,
diseases (for example, systemic lupus erythematosus), malig- Long Island Campus of the Albert Einstein College of Medicine, New
nancies (for example, leukaemia, lymphoma, angioblastic York, USA
lymphadenopathy), infections (for example, viral, tuberculo- Correspondence and reprint requests to: Dr Leonard J Rossoff, Division of
sis, Lyme disease, syphilis), as well as granulomatous Pulmonary and Critical Care Medicine, Long Island Jewish Medical
disorders (for example, sarcoidosis). Center, Long Island Campus of the Albert Einstein College of Medicine,
Room C-20, 270–05 76th Avenue, New Hyde Park, NY 11042, USA;
The diagnosis is one of exclusion and is based on clinical lrossoff@lij.edu
signs, symptoms, and laboratory findings. The diagnosis is
established by three major and two minor criteria.10 The major Submitted 22 June 2001
Accepted 24 July 2001
criteria are: 1, fever greater than 39°C, with a spiking pattern
once daily; 2, arthralgia or arthritis; 3, negative antinuclear REFERENCES
antibodies and rheumatoid factor. The minor criteria are: 1, 1 Uson J, Pena JM, Del Arco A, et al. JJ. Still’s disease in a 72-year-old
leucocyte count greater than 15 × 109/l; 2, Still’s pathogno- man. J Rheumatol 1993;20:1608–9.
2 Cheema GS, Quismorio FP Jr. Pulmonary involvement in adult-onset
monic rash (85% of patients) which is maculopapular, mildly Still’s disease. Curr Opin Pulm Med 1999;5:305–9.
pruritic, salmon pink, and found on the trunk and proximal 3 Hirohata S, Kamoshita H, Taketani T, et al. Adult Still’s disease
extremities and precipitated by mechanical irritation (Koeb- complicated with adult respiratory distress. Arch Intern Med 1986;146:
2409–10.
ner’s phenomenon); 3, pleuritis or pericarditis; 4, hepatome- 4 Jottrand M, Lienart F, Decaux G. Manifestations inhabituelles de la
galy, splenomegaly or generalised lymphadenopathy. Our maladie du Still de l’adulte. A propos de deux observations. Rev Med
patient met all three of the major criteria and three of four Brux 1985;6:699–704.
5 Paccalin M, Chapon C, Roblot P, et al. Severe pulmonary manifestations
minor criteria. in adult-onset Still’s disease. Rev Med Interne 1997;18:575–7.
Adult Still’s disease generally has a favourable prognosis, 6 Ohta A, Yamaguchi M, Kaneoka H, et al. Adult Still’s disease: review of
228 cases from the literature. J Rheumatol 1987;14:1139–1146.
however, studies have shown a slightly increased incidence of 7 Shimomoto H, Imaizumi K, Mizoguchi K, et al. A case of adult Still’s
premature death.9 Causes of death include hepatic failure, dis- disease with severe pulmonary complications. Nihon Kokyuki Gakkai
seminated intravascular coagulation, amyloidosis, and sepsis.9 Zasshi 1989;27:1092–9.
8 Suyama T, Yokoyama K. Interstitial pneumonitis detected by
Pulmonary manifestations include pleuritis, transient radio- bronchoalveolar lavage and transbronchial lung biopsy in adult-onset
logical infiltrations, interstitial lung disease, and rare respira- Still’s disease. Nihon Kokyuki Gakkai Zasshi 1998;36:545–50.
tory failure requiring mechanical ventilation.2 Pulmonary 9 Esdaile JM. Adult Still’s disease. In: Klippel JH, ed. Primer on rheumatic
diseases. 11th Ed. Atlanta: Arthritis Foundation, 1997.
manifestations have, in general, a good prognosis but there are 10 Masson C, Le Loet X, Liote F, et al. Comparative study of six types of
no published data on mortality or prognosis.2 criteria in adult Still’s disease. J Rheumatol 1996;23:495–7.

www.postgradmedj.com
 Downloaded from http://pmj.bmj.com/ on April 7, 2015 - Published by group.bmj.com

Adult Still's disease and respiratory failure in a

74 year old woman
G S Stoica, R I Cohen and L J Rossoff

Postgrad Med J 2002 78: 97-98

doi: 10.1136/pmj.78.916.97

Updated information and services can be found at:

http://pmj.bmj.com/content/78/916/97

These include:

References This article cites 9 articles, 0 of which you can access for free at:
http://pmj.bmj.com/content/78/916/97#BIBL

Email alerting Receive free email alerts when new articles cite this article. Sign up in the
service box at the top right corner of the online article.

Topic Articles on similar topics can be found in the following collections

Collections Histopathology (8)
Clinical diagnostic tests (352)
Connective tissue disease (42)
General surgery (149)
Immunology (including allergy) (346)
Radiology (364)
Rheumatoid arthritis (17)
Surgical diagnostic tests (145)

Notes

To request permissions go to:

http://group.bmj.com/group/rights-licensing/permissions

To order reprints go to:

http://journals.bmj.com/cgi/reprintform

To subscribe to BMJ go to:

http://group.bmj.com/subscribe/