Pituitary involvement in patients with granulomatosis with polyangiitis: case series and literature review

Gu, Yu; Sun, Xuguo; Peng, Min; Zhang, Ting; Shi, Juhong; Mao, Jiangfeng

doi:10.1007/s00296-019-04338-0

Cited by 14 publications

(12 citation statements)

References 36 publications

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“…In this study, only 2% AAV patients (3/150) could be confirmed as having pituitary involvement. A recently published study showed the incidence rate of pituitary involvement in GPA was 3.9%, 11 which was slightly higher than our data. A retrospective series of 819 GPA patients reported pituitary involvement in 1.1%.…”

Section: Discussioncontrasting

confidence: 85%

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Pituitary dysfunction in patients with ANCA associated vasculitis: prevalence, presentation, and outcomes

Zou

Lü

et al. 2020

Therapeutic Advances in Chronic Disease

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Background: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are rare multisystem autoimmune diseases characterized by inflammatory cell infiltration causing necrosis of small blood vessels. Pituitary involvement in AAV is poorly described. This study aimed to describe the prevalence, clinical characteristics, and outcomes of pituitary involvement in patients with AAV. Methods: A total of 150 patients diagnosed with AAV and hospitalized in the China–Japan Friendship Hospital between 2009 and 2019 were enrolled in this retrospective study. Patients diagnosed with pituitary involvement in AAV were selected for inclusion. Results: Three patients (2%) were identified with pituitary involvement. Two patients had positive ANCA titers, one with proteinase 3 positive and one with myeloperoxidase positive antibodies. Pituitary dysfunction presented as an initial symptom in one patient and developed over the course of the diseases in the other two patients. All three patients had abnormal hormones. Among them, two patients had an enlarged pituitary, shown by magnetic resonance images (MRIs), and one patient had a normal sized pituitary, shown by MRI, but presented with increased linear radioactivity uptake in the pituitary fossa by positron emission tomography-computed tomography. All patients were treated with corticosteroid and immunosuppressive therapy. Both pituitary dysfunction and vasculitis were in remission. Conclusion: Pituitary involvement is uncommon in AAV and it can occur at any point during AAV. The main clinical manifestations are central diabetes insipidus and panhypopituitarism. Immunosuppressive therapy could significantly alleviate clinical symptoms as well as pituitary imaging.

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Section: Discussioncontrasting

confidence: 85%

“…8 Two retrospective cohort studies and dozens of case reports or series about pituitary involvement with GPA were reported. [9][10][11][12][13] In this study, we described the prevalence, presentation, and outcomes of pituitary involvement in AAV.…”

Section: Introductionmentioning

confidence: 99%

Pituitary dysfunction in patients with ANCA associated vasculitis: prevalence, presentation, and outcomes

Zou

Lü

et al. 2020

Therapeutic Advances in Chronic Disease

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“…Granulomatosis with polyangiitis hypophysitis is rare (< 1%) and is usually found in association with multisystem disease (ear, nose, and throat disease and lung, as well as kidney, skin, eyes, and arthralgias) ( 74 , 75 ). However, pituitary involvement as an initial presentation was noted in almost half of cases ( 75 ).…”

Section: Specific Hypophysitis Typesmentioning

confidence: 99%

“…However, pituitary involvement as an initial presentation was noted in almost half of cases ( 75 ). There is a predilection in young females, and DI is almost universally present ( 74 , 75 ). First-line treatment is GCs; other treatment options include immunosuppressive agents such as cyclophosphamide ( 74 , 75 ).…”

Section: Specific Hypophysitis Typesmentioning

confidence: 99%

Hypophysitis, the Growing Spectrum of a Rare Pituitary Disease

Langlois

Varlamov

Fleseriu

2021

The Journal of Clinical Endocrinology &Amp; Metabolism

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Hypophysitis is defined as inflammation of the pituitary gland that is primary or secondary to a local or systemic process. Differential diagnosis is broad (including primary tumors, metastases, and lympho-proliferative diseases) and multifaceted. Patients with hypophysitis typically present with headaches, some degree of anterior and/or posterior pituitary dysfunction, and enlargement of pituitary gland and/or stalk, as determined by imaging. Most hypophysitis causes are autoimmune, but other etiologies include inflammation secondary to cellar tumors or cysts, systemic diseases, and infection or drug-induced causes. Novel pathologies such as IgG4-related hypophysitis, immunotherapy-induced hypophysitis and paraneoplastic pituitary-directed autoimmunity are also included in a growing spectrum of this rare pituitary disease. Typical magnetic resonance imaging reveals stalk thickening and homogenous enlargement of the pituitary gland, however, imaging is not always specific. Diagnosis can be challenging and ultimately, only a pituitary biopsy can confirm hypophysitis type and rule out other etiologies. A presumptive diagnosis can be made often without biopsy. Detailed history and clinical examination are essential, notably for signs of underlying etiology with systemic manifestations. Hormone replacement and in selected cases careful observation is advised with imaging follow-up. High-dose glucocorticoids are initiated mainly to help reduce mass effect. A response may be observed in all auto-immune etiologies, but also in lymphoproliferative diseases, and as such should not be used for differential diagnosis. Surgery may be necessary in some cases to relieve mass effect and allow a definite diagnosis. Immunosuppressive therapy and radiation are sometimes also necessary in resistant cases.

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“…Peripheral Nervous System (PNS) involvement is noted in approximately 15 percent of patients with GPA, and Central Nervous System (CNS) involvement is much less common [3]. There are several case reports and experiences regarding pituitary involvement in GPA [4][5][6][7]. In addition, pituitary involvement leading to Central Diabetes Insipidus (CDI) is extremely uncommon and is generally thought to occur later in the disease course.…”

Section: Introductionmentioning

confidence: 99%

Diabetes insipidus as the first manifestation of granulomatosis with polyangiitis: A case report

Behdadnia¹,

Jeddi²,

Estedlal³

2021

J Clin Images Med Case Rep

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Background: Granulomatosis with Polyangiitis (GPA) is a necrotizing granulomatosis vasculitis that may influence most organs, but posterior pituitary involvement as the first manifestation is unusual. However, there are some case reports in this regard. Case presentation: A 49-year-old menopausal woman presented with polyuria, polydipsia and weight loss over time headache, purulent nasal discharge, malaise, ear pain developed. After right myringotomy due to secretory otitis media and nasal cavity biopsy, the symptoms exacerbated. Diabetes insipidus was documented by Deprivation test. Thus, 20 microgram nasal spray DDAVP was prescribed. Pituitary MRI +/- Gad demonstrated pituitary involvement. Pituitary axis hormone evaluation was consistent with menopausal state. Chest spiral CT scan showed speculated border soft tissue mass lesion. GPA confirmed with ACR criteria and C-ANCA positive. Therefore, corticosteroid and rituximab were started. Symptoms of DI responded well to the treatment and the spray dosage was decreased. Conclusion: DI can be the first manifestation of GPA; although it is rare, it is worth to be diagnosed early and serious treatment of vasculitis should be started to prevent irreversible damage to the hypophysis cell and unnecessary procedure. Keywords: GPA; granalomatosis vasculitis; diabetes insipidus.

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Pituitary involvement in patients with granulomatosis with polyangiitis: case series and literature review

Cited by 14 publications

References 36 publications

Pituitary dysfunction in patients with ANCA associated vasculitis: prevalence, presentation, and outcomes

Pituitary dysfunction in patients with ANCA associated vasculitis: prevalence, presentation, and outcomes

Hypophysitis, the Growing Spectrum of a Rare Pituitary Disease

Diabetes insipidus as the first manifestation of granulomatosis with polyangiitis: A case report

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