Postinfantile giant cell hepatitis in the setting of autoimmune hepatitis: exclusively a histological pattern or a prognosis predictor?

Autoimmune hepatitis (AIH) is a rare chronic liver disease with a non-specific clinical presentation. Its physiopathology is not fully understood and, if untreated, can progress to cirrhosis and even fulminant liver failure. Here, we describe a case of a 73-year-old patient with an 11-month history suggestive of liver disease, who was concomitantly diagnosed with AIH and the extremely rare postinfantile giant cell hepatitis (PIGCH). Despite standard immunosuppressive therapy, the patient presented a severe clinical course, culminating in acute-on-chronic liver failure and death. This case reminds physicians of the importance of an early diagnosis, close monitoring and timely treatment of AIH. It also highlights the significant role in prognosis of the specific histological pattern of PIGCH, which has been mainly associated with a serious clinical outcome and unpredictable response to immunosuppressive therapy. Triggers of both AIH and PIGCH, such as viral infections, must be excluded, given their treatment implications.

Acute intermittent porphyria: analgesia can be dangerous.

Acute intermittent porphyria (AIP) is a rare condition, a metabolic disorder of the haem biosynthesis. An acute crisis of AIP can present as a combination of symptoms, such as abdominal pain, autonomic dysfunction, hyponatremia, muscle weakness and neurological symptoms in the absence of others obvious causes. We report the case of a 53-year-old woman, who was previously diagnosed with AIP 5 weeks after therapeutic suspension has developed an acute disease exacerbation. During hospitalisation, further exacerbation has occurred after analgesia with metamizole. Glucose and hemin infusions resulted in slow improvement. Physical rehabilitation was crucial to peripheral polyneuropathy recovery. Taking into account the porphyrinogenic effect described for metamizole, this drug might have triggered the second attack. Clinical history was sufficient to suspect the diagnosis and to start the treatment immediately, preventing important sequelae.

Pulmonary Sarcoidosis in Behçet's Disease Treated with Adalimumab.

TNF-α antagonists are used to treat various rheumatic diseases including sarcoidosis. However, there have been increasing reports of sarcoidosis in relation to treatment using these drugs. The pathogenesis of this reaction remains unknown. This is a report of a clinical case of sarcoidosis in Behçet's disease (DB) with mucocutaneous and intestinal involvement in treatment using adalimumab, with improvement after anti-TNF suspension and corticosteroid therapy. LEARNING POINTS: This clinical case demonstrates the efficacy of the anti-TNFa adalimumab in the treatment of Behçet with intestinal manifestations and not responsive to other therapeutics.To our knowledge it is the first time it is described a case of sarcoid reaction in a patient with Behçet treated with adalimumab.It shows how two complications of the use of immunosuppressants (sarcoid reaction and Legionella pneumophila pneumonia) in the same patient can difficult the correct diagnosis because of the many and overlapping clinical manifestations.

Behçet's disease--a contemporary review.

Behçet's disease (BD) is a systemic vasculitis disorder of unknown etiology, characterized by relapsing episodes of oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions. It can affect other systems including vascular, gastrointestinal and neurological systems. It occurs most frequently in an area that coincides with the Old Silk Route (between latitudes 30 degrees and 45 degrees north in Asia and Europe). BD is slightly more frequent and has a worse clinical course in men. It is believed to be due to an auto-immune process triggered by an infectious or environmental agent in a genetically predisposed individual. HLA-B51 is the most strongly associated risk factor. The International Study Group (ISG) for Behçet's Disease created a set of criteria for the diagnosis of BD. Available treatments include corticosteroids, azathioprine, cychlophosphamide, cyclosporine A, interferon-alpha, anti-tumour necrosis factor alpha agents, among others. BD has a variable course characterized by relapses and remissions. Prognosis depends on the clinical involvement. Loss of visual acuity and neurological disease are major causes of morbidity and disability.

Miliary tuberculosis and Q fever in an immunocompetent patient.

Miliary tuberculosis is a progressive disease resulting from the massive hematogeneous dissemination of Mycobacterium tuberculosis. Immunocompromised and elderly patients are the most affected. A case of an immunocompetent 45 year old man, with miliary tuberculosis and a simultaneous infection by Coxiella burnetti is presented. The clinical presentation demands a high awareness for the presence of tuberculosis. The rarity of this association, only one case reported in literature, appears to be a fortuity coincidence.