Discussion
We presented a young female patient who had HLH secondary to AOSD. His
clinical and laboratory findings included fever, arthralgia,
salmon-colored rash, lymphadenopathy, negative ANA, negative rheumatoid
factor. She had hemophagocytosis. So she had five out of the eight
diagnostic criteria of HLH. Then, the patient was diagnosed with HLH
followed by AOSD (6, 7).
Still’s disease is an autoimmune disorder. Still’s disease with adult
onset (AOSD) has two ranges of presentation including 15-25 and 36-46
years of age (8). The etiology of this disease is not clear. Some
factors including infections genetic factors, (viral or bacterial), and
immune disorders are suggested (8).
AOSD is characterized by fever, non-suppurative pharyngitis, transient
rash (salmon-colored, maculopapular, nonpruritic, and often observed
during febrile episodes) involving the trunk and proximal extremities,
arthralgia that commonly involving the wrists, ankles, knees, and elbows
(8). Our case had fever, pharyngitis, rashes with trunk involvement, and
arthralgia especially in phalanxes.
Laboratory findings in AOSD are similar to other autoimmune disorders.
Common laboratory findings are elevated white blood cell (WBC) count,
abnormal LFTs, and elevated levels of CRP, and ferritin. In this
disease, RF and ANA are usually negative. For diagnosis of AOSD,
physicians should exclude infections disorders, vasculitis,
malignancies, and other connective tissue disorders (8). We were
suspicious to AOSD but we also excluded malignancies and infections
disorders by different evaluations of the patient.
Of the most serious complications of AOSD is HLH. AOSD and HLH have
similar clinical findings and laboratory results. These similar features
make a diagnostic challenge for physicians (9). HLH unlike to AOSD may
have thrombocytopenia or leukopenia, and very high levels of
triglyceride in serum (10). We observed significant elevation in LFT,
LDH, Ferritin, CRP, WBC levels. Thrombocytopenia and anemia (low level
of hemoglobin) were observed in the laboratory tests of our case.
Laboratory and clinical findings accompanying with decreasing trends of
lineage blood cells suspected us to diagnose AOSD + HLH in the patient.
We found high levels of serum ferritin in our case. It seems that a key
to diagnosis AOSD is very high serum ferritin. Such a finding occurs in
AOSD with HLH (11).
The treatment of HLH are corticosteroids and cyclosporine. Non- steroid
anti inflammation drugs and corticosteroids were recommended for
treatment of AOSD. We used a combination of these treatments for our
patient and she responded to these treatments and was discharged with a
good condition.