scholarly journals Adult onset still's disease: a rare disorder

Author(s):  
Belema O. Abbey ◽  
Dasetima D. Altraide ◽  
Bolaji I. Otike-Odibi

Adult onset disease is a variant of systemic onset juvenile idiopatic arthritis characterised by seronegative poly arthritis in association with multi systemic inflammation. it is often seen in young adults. Authors have report a case of a 28 year old male who presented to us with a predominantly systemic symptoms. patient presented with a 6 month history of high grade fever, with associated recurrent joint pains, sore throat, generalized tonic-clonic seizures and skin rashes. Patient remained markedly ill for most of his in-patient stay. Aesilogy is unknown, though it is currently thought that there is interplay between a genetic predisposition, an immune dysregulation and environmental play mainly infectious. Therapeutic decisions should be based on the extent and severity of organ involvement.

2021 ◽  
Vol 8 (4) ◽  
pp. 582-584
Author(s):  
Garima Shah ◽  
Bikram Shah

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.


2010 ◽  
Vol 2 (2) ◽  
pp. 149-151 ◽  
Author(s):  
SA Inamdar ◽  
Neharika Malhotra ◽  
S Sirsam

ABSTRACT A 26 years old female G3P2L2 with a previous LSCS done 10 months back, with 15 weeks twin gestation and history of criminal abortion by quack with a wooden stick brought to us in a moribund state with complaints of high grade fever with severe pain in abdomen associated with nausea and vomiting that morning. Clinical examination revealed incomplete abortion with features of sepsis. After evaluation a diagnosis of septic abortion was established based on the clinical presentation and biochemical parameters. This case is being reported for the increased incidence of illegal unsafe abortions in rural India despite legalizations of abortion.


2020 ◽  
pp. 1-2
Author(s):  
Showkat Nazir Wani ◽  
Anish Garg Atul Kaushik ◽  
Atul Kaushik ◽  
Naveen Tariq

We report a case of dengue fever with features of encephalitis from Northern India. A 22 years female presented with history of high-grade fever with chills for 2 days followed by altered sensorium and MRI brain, revealed characteristic changes consistent with Encephalitis with hemorrhage mainly involving the bilateral thalami. The diagnosis of dengue was confirmed by the presence of dengue antigen in the serum and the presence of dengue antibodies in the serum as well as cerebrospinal fluid. Dengue is not primarily a neurotropic virus and encephalopathy is an uncommon finding in dengue. This case explains the importance of considering dengue encephalitis as the differential diagnosis of fever with altered sensorium, especially in countries like India where dengue is rampant.


2009 ◽  
Vol 16 (02) ◽  
pp. 302-304
Author(s):  
MUHAMMAD SHAHID AZIZ

A young soldier 20 years of age was brought with 02hour history of sudden loss of consciousness associatedwith high grade fever while working in the field for severalhours in hot and humid weather in month of August.There was no history of head trauma.


2018 ◽  
Vol 29 (2) ◽  
pp. 91-93 ◽  
Author(s):  
Homayra Tahseen Hossain ◽  
Quazi Tarikul Islam ◽  
Ishrat Binte Reza ◽  
Tasmina Chowdhury ◽  
Md Maksudul Mazumder ◽  
...  

DRESS syndrome (Drug reaction with Eosinophilia and Systemic Symptoms) is a rare but potentially life-threatening drug hypersensitivity reaction characterized by fever, exfoliative dermatitis and maculopapular rash, lymphadenopathy, eosinophilia, leucocytosis, and involvement of internal organs as liver, lung, heart, and kidney. The disorder starts within 2–8 weeks after taking an offending drug. Long latency between drug treatment and onset of symptoms along with variability of presentation make the diagnosis quite difficult. Here, we are presenting a case report of 58 year old male presented with short history of high grade fever with extensive skin rash. Investigations revealed leucocytosis with gross eosinophilia. He gave history of taking carbamazepine for more than last 3 months prescribed by his neurologist. According to the RegiSCAR scoring system, our case could be classified as definite DRESS syndrome. After withdrawal of the offending drug carbamazepine and starting high dose of systemic corticosteroid, he improved dramatically. As this syndrome is quite uncommon, so we are reporting this case.Bangladesh J Medicine Jul 2018; 29(2) : 91-93


2018 ◽  
Vol 36 (3) ◽  
pp. 123-127
Author(s):  
Safia Binte Rabbani ◽  
Md Iqbal Hossain ◽  
Afsana Begum

Scrub typhus is an acute, febrile, infectious illness that is caused by Orientia tsutsugamushi. Scrub and murine endemic typhus infections are under-diagnosed causes of febrile illness across the tropics, and it is not known how common they are in Bangladesh. Here reporting a case who presented with high grade fever for 7 days associated with headache, abdominal discomfort, cough and receiving inj ceftriaxone for 2 days considering as a case of enteric fever. Forty-eight hours after admission, the presence of continued high grade fever, conjunctival congestion, headache, loose motion, cough, low platelet, elevated transaminase raised the possibility of a different aetiology. The two most likely differentials being dengue and malaria. Finally, typhus was discovered due to history of field visit, presence of eschar and raised titre of WF OXK antigen. All other causes of fever were ruled out by history, clinical examination and relevant investigations. Patient was treated with capsule doxycycline and other supportive therapy and become afebrile within 48 hours. This uncommon clinical scenario should be taken into account in the management of patients with high fever on admission.J Bangladesh Coll Phys Surg 2018; 36(3): 123-127


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Natalia Cernovschi - Feasey ◽  
Julekha Wajed

Abstract Background/Aims  Dactylitis is commonly associated with psoriatic arthritis, and regularly presents at Rheumatology clinics. We discuss a case where progressive systemic symptoms lead to the consideration of alternate diagnoses. Methods  A 46-year-old Nepalese woman presented to the Rheumatology department with a 3 month history of diffuse swelling of the right middle finger proximal interphalangeal joint, with the appearance of dactylitis. There was pain on movement, but no other joint involvement. Simultaneously she noticed blurred and decreased vision, which on review by the ophthalmologists, was diagnosed with bilateral uveitis. There was no history of psoriasis, inflammatory bowel disease, or other past medical history of note. There was no travel history in the past 12 months. A diagnosis of a presumed inflammatory arthritis was made. Results  Blood tests showed elevated c-reactive protein 55 (normal <4 mg/l), erythrocyte sedimentation rate 138 (normal 0-22 mm/hr) and an iron deficiency anaemia. Rheumatoid factor and Anti-CCP antibody were negative. Hand radiographs were reported as normal. MRI of the third digit confirmed an enhancing soft tissue collection at the proximal phalanx of the right middle finger. She was referred for a biopsy of this lesion. Interestingly over the subsequent few months, she developed progressive breathlessness. Chest radiograph showed a left pleural effusion. Further tests showed negative serum ACE, Lyme and Toxoplasma screen. Quantiferon test was negative. Pleural aspirate showed a transudate with negative Acid-fast bacillus (AFB) test and culture. CT chest and abdomen showed a persistent pleural effusion, inflammatory changes in the small bowel and thickening of the peritoneum and omentum. In view of the systemic involvement, a peritoneal tissue biopsy was performed. This confirmed chronic granulomatous inflammation with positive AFB stain for mycobacterium tuberculosis. Our patient was started on quadruple anti- TB antibiotics for 6 months. Her systemic symptoms and dactylitis have improved, although there is on-going treatment for her ocular involvement. Conclusion  Approximately 10% of all cases of extrapulmonary TB have osteoarticular involvement. Dactylitis is a variant of tuberculous osteomyelitis affecting the long bones of the hands and feet. It occurs mainly in young children; however adults may be affected also. The first manifestation is usually painless swelling of the diaphysis of the affected bone followed by trophic changes in the skin. The radiographic changes are known as spina ventosa, because of the ballooned out appearance of the bone, although this was not seen in our case. Fibrous dysplasia, congenital syphilis, sarcoidosis and sickle cell anaemia may induce similar radiographic changes in the metaphysis of long bones of hands and feet, but do not cause soft tissue swelling or periosteal reaction. This case highlights the importance of testing for TB, especially in atypical cases of dactylitis, with other systemic features. Disclosure  N. Cernovschi - Feasey: None. J. Wajed: None.


2020 ◽  
Vol 24 (3) ◽  
pp. 267-272
Author(s):  
Airiss R. Chan ◽  
Vijay K. Sandhu ◽  
Aaron M. Drucker ◽  
Patrick Fleming ◽  
Charles W. Lynde

Atopic dermatitis (AD) is a chronic skin disease characterized by barrier dysfunction and immune dysregulation that affects approximately 20% of children and 2-5% of adults worldwide. Traditionally, AD has been considered a disease of childhood with many cases resolving before adulthood. However, in recent years, the prevalence of adult AD is increasingly recognized to be substantial, but it is uncertain whether this increase is due to increased childhood-persistent or relapsed AD, or new adult-onset AD. This highlights a need for further investigation into the adult AD population and evaluation of phenotypes in the adult-onset cohort. In this literature review, we examine five studies focused on adult-onset AD phenotype, conducted between 2013 and 2017. The most commonly reported body regions affected in adult-onset AD were the hands, eyelids, neck, and flexural surfaces of the upper limbs. These vary from childhood-onset AD findings, which are less specific to body regions other than flexural areas. These findings have implications for diagnostic accuracy and treatment of AD, including considerations for therapeutic choices and inclusion and exclusion criteria in clinical trials.


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