Clinical Brief

SLE Presenting as Epistaxis

Mathew Pathrose Kallingal

Department of Pediatrics, Ministry of Health, Sultanate of Oman

ABSTRACT

Epistaxis as a presenting complaint in systemic lupus erythematosus is very rare. An 11-yr-old girl presented with an episode
of profuse epistaxis, self limiting, and was seen to be anemic with deranged coagulation profile. Subsequent work up showed
the child to have thrombocytopenia, and she had blood and platelet transfusion. However, the epistaxis of the
thrombocytopenia persisted, despite the corrective measures. Investigations for the cause of thrombocytopenia, lead to the
discovery of laboratory features, suggestive of systemic lupus erythematosus. [Indian J Pediatr 2010; 77 (5) : 561-562]
E-mail mathewkallingal@yahoo.com

Key words: Epistaxis; Systemic lupus erythematosus; Child

Systemic lupus erythematosus (SLE) is a rheumatic showed low platelets and no blast cells, which was further
disease, of unknown cause, characterized by corroborated by bone marrow study which showed active
autoantibodies directed against self-antigens, resulting in marrow and reduced platelets
inflammatory damage to target organs.1
Based on these findings, treatment for autoimmune
Epistaxis is a common complaint in any pediatric idiopathic thrombocytopenia was initiated. The child had
setting, and evaluation of such a minor complaint may be blood and platelet transfusion and was commenced
a worthwhile pursuit, as this case illustrates. What started adjunct with oral steroids (40mg prednisolone three times
as a first time epistaxis, ended up as a case of SLE. a day). However, the epistaxis persisted for more than
two days with bleeding from injection sites, even after the
initiation of the treatment. The only cause of bleeding was
REPORT OF CASE
found to be thrombocytopenia, all other parameters being
normal as per subsequent evaluation. The cause of the
An 11-yr-old Omani girl presented in a primary health thrombocytopenia remained elusive until the elucidation
care facility with severe bleeding from the nose, which of laboratory features suggestive of SLE (Table1). The
was self limiting. There was no history of bleeding in the child was discharged on prednisolone 40mg, three times
past or any bleeding disorder in the family. On a day. A few wk later the child presented with occipital
examination, the child had fever (39oC) and appeared to
be very pale. Her blood pressure was normal for her age TABLE 1. Laboratory Findings
and height. She was pre-pubertal in development with no
other significant clinical findings. An initial blood work Parameters Patient Value Normal range
up showed profound low hemoglobin (7.6g/dl), bleeding Hemoglobin (g/dl) 6.6 11.4 - 14.5
time of 8 min and prothrombin time of 24 sec (control, 14 Platelets 17.5×109 150-400×109
sec). She had self limiting episodes of recurrent epistaxis IgG (g/L) 29.3 6.5 - 16
later. The child was further evaluated in a secondary IgA (g/L) 3.5 0.4 - 2.5
facility where the primary findings were anemia and IgM (g/L) 2.03 0.5 - 2.7
ANA Strongly Positive
thrombocytopenia (Table 1). A peripheral blood film
C 4 (mg/L) 62 160 - 380
C 3 (mg/L) 449 790 - 1520
Correspondence and Reprint requests : Dr. Mathew Pathrose Anticardiolipin IgG (u/ml) 18.4 0.1 - 15
Kallingal, MBBS, Dch, Junior specialist in pediatrics, Bidaya Health Anti ds DNA Positive
center, P.O.BOX 380, Post code 316 Ministry of Health, Oman. Epstein Barr virus IgG Positive

[DOI-10.1007/s12098-010-0069-2] ANA-Anti nuclear antibodies, Ig-Immunoglobulin, C- Comple-

[Received April 28, 2009; Accepted January 25, 2010] ment, ds - double stranded

Indian Journal of Pediatrics, Volume 77—May, 2010 561

 Mathew Pathrose Kallingal
scalp abscess, which was draining. A check on the blood
sugar revealed high values (29 mmol/L). Pus culture
from the abscess revealed methicillin resistant
staphylococci for which hospital stay was attributed.
Insulin therapy was started for diabetes mellitus and the
child was placed on a regular review. The steroids were
tapered off over a period of time and replaced with
Azathioprine. Subsequently, over the next few month, the
child had the following significant developments: Insulin
was weaned off; the child had arthralgia of the wrist, knee
and ankle joints and the Anti ds DNA disappeared.
DISCUSSION
The presence of four or more criteria is compatible with a
diagnosis of SLE, with a sensitivity and specificity of 75%
and 95%, respectively.2 However, these criteria may be
present serially or simultaneously, and current literatures
are advocating treatment as for SLE even if less than the
four of the eleven criteria have been satisfied as in this
case.1, 2 Epistaxis complicating SLE is very rare, still rarer
is epistaxis as a presenting complaint in SLE.1
Bach had reported a case of epistaxis as a presenting
compliant in nasal perforation complicating SLE. 3
Engelsen et al had reported a case of epistaxis in a 15-yr-
old girl with menorrhagia, the hemorrhagic manifestation
was attributed to lupus anticoagulant, complicated by
hypoprothrombinaemia resulting from prothrombin
antibodies. 4 Rodriguez et al had reported a case of
epistaxis in a 16-yr-old boy with lupus anticoagulant-
antiphospholipid syndrome; however it was complicated
by hematuria and gastrointestinal bleeding.5
Sumboonnanonda et al had reported epistaxis in 1.4%
of children who had hypertension complicating SLE.6
Poor prognostic factors leading to approximately 50%
mortality in 10 years, seen in this case include anemia at
presentation, hypocomplementemia, thrombocytopenia
and low socioeconomic status.2 This further justifies the
commencement of therapy despite the absence of
necessary criteria, initially, to make a diagnosis of SLE.
The hyperglycemia could be attributed to the steroids;
562
hence insulin was tapered off with the change of therapy
to Azathioprine. The disappearance of Anti ds DNA can
be attributed to therapy as it is a serological marker of
disease activity.1 A suspected trigger of disease can be
attributed to Epstein Barr virus, as Epstein Barr virus
Immunoglobulin were found positive in the child.2
This case illustrates the varied presentations of SLE.
Early diagnosis and treatment of SLE can improve the
prognosis, of what used to be a fatal disease.1
Acknowledgements
I acknowledge with thanks, the help rendered by Dr Promod
Kumar, specialist in Dermatology, Saham hospital, North Batinah,
Sultanate of Oman, in preparing the manuscript.
Contributions: MPK; the sole author, the case presented in his clinic
and was followed up.
Conflict of Interest: None.
Role of Funding Source: None.
REFERENCES
1. Klein-Gitelman MS, Miller ML. Systemic Lupus
Erythematosus. In Behrman, Kliegman, Jenson, Stanton, ed.
Nelson Textbook of Pediatrics, 18th ed. (Indian Edition), New
Delhi; Elsevier, 2008; 1015-1019.
2. Hahn BH. Systemic Lupus Erythematosus. In Fauci,
Braunwald, Kasper, Hauser, Longo, Loscalzo et al ed.
Harrison's Principles of Internal Medicine, 17th ed. (Indian
edition), New Delhi; McGraw - Hill, 2008; 2075-2084.
3. Bach GL. Nasal septum perforation in disseminated lupus
erythematosus, a case report and literature review.
Z Rheumatol 1980; 39: 22-27.
4. Engelsen J, Nielsen SM, Thorsen S. Serious bleeding in
systemic lupus erythematosus complicated by lupus
anticoagulant -hypoprothrombinaemia syndrome. Ugeskr
laeger 2006; 168: 486-487.
5. Rodriguez V, Reed AM, Kuntz NL, Anderson PM, Smithson
WA, Nichols WL. Antiphospholipid syndrome with
catastrophic bleeding and recurrent ischemic strokes as an
initial presentation of systemic lupus erythematosus. J Pediatr
Hematol Oncol 2005; 27: 403-407.
6. Sumboonnanonda A, Chongcharoensuk C, Supavekin S,
Pattaragran. Persistent hypertension in Thai children:
Etiologies and Outcome. J Med assoc Thai 2006; 89 suppl 2: S28-
S32.
Indian Journal of Pediatrics, Volume 77—May, 2010