Indian Journal of Rheumatology 2007 June

PG Forum
Volume 2, Number 2; p. 76

Rheumatology quiz
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V Arya1, V Dhir2
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1. The genetic marker most consistently associated with 6. In Kawasaki disease

Behcet’s disease is? (a) Coronary involvement is seen in majority of
(a) HLA B 15 patients
(b) HLA B 51 (b) Intravenous immunoglobulin has no role
(c) HLA B 25 (c) Thrombocytopenia is common
(d) HLA B 21 (d) Aspirin is indicated indefinitely in those with coro-
2. Pleural effusions associated with RA are characterized nay aneurysms
by all the following, except 7. Milwaukee shoulder is characterized by:
(a) Rare bilaterality (a) Greatest incidence in young men
(b) Exudative fluid (b) Non-erosive arthritis
(c) Very low glucose levels (c) Large joint effusions
(d) Very high LDH levels (d) Inflammatory joint fluid
3. Scleromalacia perforans, a rare complication, is charac- 8. Post-streptococcal reactive arthritis differs from acute
teristically seen in patients with: rheumatic fever in all the following, except
(a) Ankylosing spondylitis (a) Higher prevalence in males
(b) Psoriatic arthritis (b) Persistence of arthritis beyond 4 weeks
(c) Rheumatoid arthritis (c) Preponderance in older individuals
(d) Sarcoidosis (d) Poor response to salicylates
4. Renal biopsy in patients with RA may show any of the 9. Multicentric reticulohistiocytosis differs from RA in
following, except having
(a) Membranous GN (a) Non-erosive arthritis
(b) Interstitial nephritis (b) Distal interphalangeal joint involvement
(c) Amyloid deposits (c) Asymmetrical joint involvement
(d) Crescentic GN (d) Male predominance
5. Posterior uveitis is characteristically seen in: 10. Thyroid acropachy is characterized by all, except
(a) Ankylosing spondylitis (a) Clubbing
(b) Behcet’s disease (b) Periostitis
(c) Rheumatoid arthritis (c) Painful swelling of fingers and toes
(d) Psoriatic arthritis (d) Equally common in males and females
For answers refer to page 78

1
Clinical Immunology and Rheumatology Service, Department of Medicine, All India Institute of Medical Sciences, New Delhi, 2Department of
Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Correspondence: Dr. V Arya, email: linuxphoenix@gmail.com
 78 Indian Journal of Rheumatology 2007 June; Vol. 2, No. 2
ANSWER
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The above case history was typical of diffuse alveolar
haemorrhage (DAH). Fall in the haemoglobin level from 11
to 6 gm/dl and bilateral pulmonary infiltrates on chest X-
ray were strong pointers towards this diagnosis. A CECT
nYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8K2+Ya6H515kE= on 03/17/2023
thorax showed findings consistent with DAH. Bronchoalve-
olar lavage (BAL) fluid further confirmed the diagnosis with
the presence of haemosiderin laden macrophages. Patient
was treated with injection methylprednisolone pulses for
3 days along with a broad spectrum antibiotic cover, oxygen
therapy, blood transfusion, and other supportive therapy.
Within 48 hours, dyspnoea and haemoptysis subsided
completely.
DAH complicating systemic lupus erythematosus (SLE)
is a devastating condition. It is an uncommon medical emer-
gency with a high mortality and requires a high index of
suspicion for diagnosis. In one such study, it accounted for
3.7% of the 510 admissions due to various complications of
SLE.1 It can occur in a number of medical conditions, the
principal amongst which are isolated pauci-immune capil-
laritis, pulmonary allograft rejection, SLE, Wegener’s granu-
lomatosis, microscopic polyangitis, Goodpasture’s syndrome,
rheumatoid arthritis (RA), polymyositis and primary antiphos-
pholipid syndrome.2 It usually occurs after a median period
of 31–35 months after the diagnosis of SLE. However, in
20% of the patients it can be the initial manifestation.1
Haemoptysis, dyspnoea cough and fever are the important
initial presenting features. One-third to half of the patients
may not have haemoptysis on presentation. In these patients,
the presence of cough, dyspnoea and fall in haematocrit pro-
vide clues to the diagnosis of DAH. It has been observed that
in more than 90% of cases, DAH occurs in patients with active
ANSWERS TO THE RHEUMATOLOGY QUIZ
(page 76)
1b, 2a*, 3c, 4d**, 5b, 6d***, 7c†, 8a, 9b††, 10c†††
*
May be bilateral in about 25% of the cases.
**
Membranous GN: Gold/D-penicillamine therapy; intersti-
tial nephritis: NSAIDs; amyloid: complication of long-
standing RA.
Marwaha et al.
glomeurlonerphritis.1 Fifty percent of patients with DAH may
require assisted ventilation2 and the mortality is around 50%.
Lung biopsy shows small vessel vasculitis in the form of
arteriolitis, venulitis, and capillaritis.1,3,4 In addition, increased
CO transfer on diffusion studies and haemosiderin-laden
macrophages on BAL fluid confirm the diagnosis. The treat-
ment of DAH comprises intravenous high-dose steroids.2,5
Following a therapeutic response, corticosteroids are tapered
over a period of 2–6 months. Use of cyclophosphamide and
the other immunosuppressive agents like azathioprine is dic-
tated by the presence of other indications such as lupus
nephritis or CNS involvement.
REFERENCES
1. Zamora MR, Warner ML, Tuder R, Schwarz MI. Diffuse
alveolar hemorrhage and systemic lupus erythematosus
(SLE): clinical presentation, histology, survival and outcome.
Medicine 1997; 76: 192–202.
2. Schwartz MI, Brown KK. Small vessel vasculitis of lung.
Thorax 2000; 55: 502–10.
3. Abud-Mendoza C, Diaz-Jouanene E, Alarcon-Segovia D.
Fatal pulmonary haemorrhage in systemic lupus erythemato-
sus. Occurrence without hemoptysis. J Rheumatol 1985; 12:
558–61.
4. Myers JL, Katzenstein ALA. Microangitis in lupus induced
pulmonary haemorrhage. Am J Clin Pathol 1986; 85: 553–6.
5. Hansen T, Brockmann H, Gaumann A, Mayet W, Scwarting A,
Galle PR, et al. Fulminant course of diffuse alveolar hemorrahge
in systemic lupus erythematosus—a case report. Z Rheumatol
2002; 61(2): 175–9.
***
Coronary involvement in only 20–25%; IvIG drug of
choice in early disease; thromboycytosis common.
†
More common in older women; destructive arthritis; joint
fluid non-inflammatory.
††
Erosive arthritis; symmetrical; female predominance.
†††
Swelling is painless.