Adult onset stills disease: Newly diagnosed in pregnancy with rare clinical presentation as pleural effusion

Abstract

Adult onset Still’ disease (AOSD) is a rare inflammatory disease which involves multiple systems and is of unknown etiology first described in 1971.It has more prevalence among young females and few studies show the exacerbation of symptoms during pregnancy. Patients usually presents with high grade fever, arthritis, arthralgia, salmon pink rash, organomegaly, lymphadenopathy but rarely can present with pleural symptoms, cardiac symptoms. Several criteria’s are used for diagnosis but Yamaguchi’s has the highest sensitivity of 93.5%. Yamaguchi’s classification criteria includes major and minor criteria’s and exclusion criteria’s. Although diagnostic criteria are there but AOSD till date is a diagnosis of exclusion. All the causes of clinical symptoms should be excluded which involves malignancies, infections, inflammatory conditions, autoimmune diseases before the diagnosis of AOSD is made. A 26-year-old female with G2P0010 and POG 6 weeks presented to medicine OPD with high grade fever associated with chills and rigors from 1 week. She was also complaining of stiffness and pain in multiple joints with chest pain, easy fatigability. She was having rashes on upper back below nape of neck. All the necessary investigations were carried out. It was found that she was also having right sided pleural effusion and severe microcytic hypochromic anemia. After batteries of investigations and consultation by obstetrician, rheumatologist, dermatologist diagnosis of Adult Onset Still’s disease was made. It was a diagnosis of exclusion. Patient was started on steroids and she responded well. All the symptoms including pleural effusion was subsided. Adult onset still’s disease is a form of still’s disease. It is a rare systemic auto-inflammatory disease. Yamaguchi is the most sensitive criteria but till date it is diagnosed after excluding possible causes of malignancies, infections and autoimmune conditions. Sometimes patients do present with rare symptoms like pleural effusion, pericarditis some may present as acute respiratory distress syndrome or mild symptoms like cough, pleuritic chest pain. In our case also all the possible causes were excluded as patient presented with pleural effusion for the first time during pregnancy, so the impact of the disease on the fetal outcome was also considered. AOSD has always been a diagnosis of exclusion but as in our case and cases with similar presentation AOSD should be consider as a differential diagnosis. Although there are only few studies which shows that there is fatal outcome of pregnancy in AOSD however there are studies showing exacerbation of symptoms of AOSD during pregnancy. Its early diagnosis and prompt treatment can be helpful in the better outcome of pregnancy as well as better prognosis of disease.