Bolesnik s adrenalnom insuficijencijom i pernicioznom anemijom: je li nova podjela autoimunog poliglandularnog sindroma primjerena?

A case of autoimmune polyglandular syndrome (APS) is presented. A 45-year-old man was admitted due to fatigue, malaise and inappetence. He had a history of primary hypothyroidism and was on levothyroxine substitution therapy. One year before, he was diagnosed with normocytic anemia and vitamin B12 deficiency, which was treated with vitamin B12 substitution therapy. Physical examination revealed hypotension and marked hyperpigmentation. Laboratory testing showed hyponatremia, hyperkaliemia and severe normocytic anemia. Endocrinological evaluation disclosed low morning cortisol and increased adrenocorticotropic hormone levels. Hence, the diagnosis of Addison’s disease was established. Additional laboratory workup showed positive parietal cell antibodies. However, his vitamin B12 levels were increased due to vitamin B12 supplementation therapy, which was initiated earlier. Gastroscopy and histopathology of gastric mucosa confirmed atrophic gastritis. Based on prior low serum vitamin B12 levels, positive parietal cell antibodies and atrophic gastritis, the patient was diagnosed with pernicious anemia. Hydrocortisone supplementation therapy was administered and titrated according to urinary-free cortisol levels. Electrolyte disbalance and red blood cell count were normalized. This case report demonstrates rather unique features of pernicious anemia in a patient with Addison’s disease. It also highlights the link between type II and type III APS. Not only do they share the same etiological factors, but also overlap in pathophysiological and clinical characteristics. This case report favors older classification of APS, which consolidates all endocrine and other organ-specific autoimmune diseases into one category. This is important since it might help avoid pitfalls in the diagnosis and treatment of patients with APS.Prikazujemo slučaj bolesnika s autoimunim poliglandularnim sindromom (APS). Muškarac u dobi od 45 godina hospitaliziran je zbog opće slabosti i malaksalosti. Od ranije je bolovao od primarne hipotireoze i uzimao je nadomjesnu terapiju levotiroksinom. Jednu godinu ranije otkrivena je normocitna anemija i deficit vitamina B12, zbog čega je liječen nadomjesnom terapijom vitaminom B12. Pri fizikalnom pregledu nađena je hipotenzija i naglašena hiperpigmentacija kože. Ulaboratorijskim nalazima nađena je hiponatremija, hiperkalemija i teška normocitna anemija. Endokrinološkom obradom nađen je snižen jutarnji kortizol te povišen ACTH, nakon čega je postavljena dijagnoza Addisonove bolesti. Dodatnom laboratorijskom obradom nađena su i pozitivna protutijela na parijetalne stanice uz povišenu koncentraciju vitamina B12 posljedično nadomjesnoj terapiji. Nalaz gastroskopije i patohistološke analize sluznice upućivao je na atrofični gastritis te je stoga postavljena dijagnoza perniciozne anemije. Započeta je nadomjesna terapija hidrokortizonom i titrirana prema ciljnim vrijednostima slobodnog kortizola u 24-satnoj mokraći. Elektrolitski disbalans i anemija su se normalizirali. Ovaj prikaz slučaja je opisao karakteristike perniciozne anemije u bolesnika s Addisonovom bolešću te naglašava vezu između APS tip II. i III. Ova dva sindroma dijele istu etiologiju te se njihove komponente često preklapaju. Slučaj našega bolesnika daje prednost starijoj klasifikaciji APS koja dopušta kombinacije svih primarnih endokrinih insufucijencija i drugih za organ specifičnih bolesti. Poštivajući staru podjelu APS mogu se izbjeći potencijalne zamke u dijagnostici i liječenju

Treatment of prolactinomas in low-income countries

Purpose. In low-income countries, prolactinomas are difficult to manage with dopamine agonists (DA). We compared the effectiveness of DA in microprolactinomas as a first line treatment and as adjuvant therapy for residual macroprolactinomas treated surgically. ----- Methods. Our retrospective study analyzed 78 patients, 38 with microprolactinomas and 40 with macroprolactinomas. Microprolactinomas were treated with DA. Macroprolactinomas were treated with microsurgical or endoscopic adenomectomies and adjuvant DA. Surgical remission was defined as normoprolactinemia three months postoperatively, and long-term remission as normoprolactinemia at the last control. ----- Results. Surgical remission was achieved in 9 patients (23%). Postsurgical tumor mass was reduced by 50% (34-68). Residual macroprolactinoma size was greater than microprolactinoma size prior to treatment (10 mm versus 4 mm, P < 0.001). Both groups received similar doses of DA. Long-term remission occurred in 68% of microprolactinomas and 43% of macroprolactinomas (P = 0.102). Prolactin (PRL) levels at the last control were similar in both groups (23.1 versus 32.9 mcg/L, P = 0.347). ----- Conclusion. Comparable remission rates and PRL levels were reached in microprolactinomas and macroprolactinomas using similar doses of DA. Although complete tumor resection is the goal of surgery, our study suggests that even partial surgical removal has a role in treatment of prolactinomas since it may enhance the response to DA

Coexistence of Addison’s Disease and Pernicious Anemia: Is the New Classification of Autoimmune Polyglandular Syndrome Appropriate?

A case of autoimmune polyglandular syndrome (APS) is presented. A 45-year-old man was admitted due to fatigue, malaise and inappetence. He had a history of primary hypothyroidism and was on levothyroxine substitution therapy. One year before, he was diagnosed with normocytic anemia and vitamin B12 deficiency, which was treated with vitamin B12 substitution therapy. Physical examination revealed hypotension and marked hyperpigmentation. Laboratory testing showed hyponatremia, hyperkaliemia and severe normocytic anemia. Endocrinological evaluation disclosed low morning cortisol and increased adrenocorticotropic hormone levels. Hence, the diagnosis of Addison’s disease was established. Additional laboratory workup showed positive parietal cell antibodies. However, his vitamin B12 levels were increased due to vitamin B12 supplementation therapy, which was initiated earlier. Gastroscopy and histopathology of gastric mucosa confirmed atrophic gastritis. Based on prior low serum vitamin B12 levels, positive parietal cell antibodies and atrophic gastritis, the patient was diagnosed with pernicious anemia. Hydrocortisone supplementation therapy was administered and titrated according to urinary-free cortisol levels. Electrolyte disbalance and red blood cell count were normalized. This case report demonstrates rather unique features of pernicious anemia in a patient with Addison’s disease. It also highlights the link between type II and type III APS. Not only do they share the same etiological factors, but also overlap in pathophysiological and clinical characteristics. This case report favors older classification of APS, which consolidates all endocrine and other organ-specific autoimmune diseases into one category. This is important since it might help avoid pitfalls in the diagnosis and treatment of patients with APS