Severe localised granulomatosis with polyangiitis (Wegener's granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review.

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Term Occurence Count Dictionary
hypogonadism 1 endocrinologydiseases
hypopituitarism 2 endocrinologydiseases
hypothyroidism 1 endocrinologydiseases
testosterone 1 endocrinologydiseasesdrugs
cortisol 8 endocrinologydiseasesdrugs
cyclophosphamide 23 endocrinologydiseasesdrugs
desmopressin 1 endocrinologydiseasesdrugs
diabetes insipidus 9 endocrinologydiseases

Graph of close proximity drug and disease terms (within 200 characters).

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Select Drug Character Offset Drug Term Instance
cortisol 24061 [[34]]7758239271*71FIntrasellar mass with suprasellar extensionDI (post-surgery)↓ TSH↓ LH & FSH↓ cortisol Normal prolactinNonecANCA +ve28*28FIntrasellar mass with low-density centreDI (post-surgery) Normal prolactin
cortisol 24211 +ve28*28FIntrasellar mass with low-density centreDI (post-surgery) Normal prolactin Normal TSHNormal LH & FSHNormal cortisol Ocular, arthralgia, cutaneous, renalcANCA +veBertken 1997 [[35]]9265867136*36FMacrocystic pituitary mass
cortisol 24956 thickening, contrast-enhancement.Loss of PSDIHyperprolactinaemiaHypogonadismNormal TSHNormal LH & FSHNormal cortisol & ACTHENT, renal, pulmonaryANCA +veGoyal 2000 [[38]]110032801N/A (many years before pituitary involvement)48FSellar
cortisol 26005 contrast-enhancement.Pituitary stalk thickened & infiltrated.Loss of PSHyperprolactinaemia↓ testosterone↓ LH, normal FSH↓ cortisol & ACTHNormal TSHConstitutional, polyarthritis, peripheral neuropathy, pulmonaryPR3 +ve4650FPituitary
cortisol 29560 peripheral enhancement. Stalk displacementDIHypogonadismNormal prolactinNormal TSHNormal IGF-1Normal cortisol ENT, pulmonary, renalN/A55MSellar mass with suprasellar extensionDIHypogonadismNormal prolactinNormal
cortisol 29680 pulmonary, renalN/A55MSellar mass with suprasellar extensionDIHypogonadismNormal prolactinNormal TSHNormal cortisol Normal IGF-1ENT, pulmonary, renal, cutaneous, jointsN/A35MNecrotic sellar mass with peripheral enhancement
cortisol 29898 suprasellar extension. Thickened contrast-enhancing stalkDI↓ TSHHypogonadismNormal prolactinNormal cortisol Normal IGF-1ENTN/A54MEnlarged pituitary measuring 12 mm, with heterogeneous enhancement. Slight diffuse
cortisol 30502 enhancement with central cystic change. Thickening of pituitary stalkDINormal prolactinNormal TSHNormal cortisol Normal IGF-1ENTDe Parisot 2015 [[19]]25906106946*46FEnlarged posterior pituitary. Infiltration of posterior
cyclophosphamide 1423 Her neurological deficits substantially improved with treatment including high dose corticosteroid, cyclophosphamide and rituximab.ConclusionsThis case emphasises that serious morbidity can arise from localised cranial
cyclophosphamide 3474 pulmonary haemorrhage or rapidly progressive crescenteric glomerulonephritis. Prior to the introduction of cyclophosphamide [[3]], mortality was over 80% [[4]]. Modern treatment involves intensive immunosuppressive therapy with
cyclophosphamide 3625 [[4]]. Modern treatment involves intensive immunosuppressive therapy with high dose corticosteroids and cyclophosphamide or rituximab to induce remission [[5], [6]]. Once remission is achieved, patients are switched to less
cyclophosphamide 9870 amenorrhoea for the past year.Fig. 2Coronal contrast-enhanced CT image of the skull base and neck (pre- cyclophosphamide treatment). There is marked abnormal chronic inflammatory infiltration of the cavernous sinuses bilaterally
cyclophosphamide 10468 and was keen to do this in the future. We therefore initially opted for treatment with rituximab over cyclophosphamide . She received Rituximab 1 g, with a plan for a second dose two weeks later. Methotrexate was replaced
cyclophosphamide 12433 assessment of response to treatment.Fig. 3Coronal T2-weighted MRI image of the skull base and neck (pre- cyclophosphamide treatment). There is severe chronic inflammatory soft tissue abnormality affecting the cavernous sinuses
cyclophosphamide 14310 (Fig. 5). Her oral prednisolone was increased to 60 mg, and she received three fortnightly doses of i.v. cyclophosphamide 750 mg (15 mg/kg) followed by three further doses at three-weekly intervals. Azathioprine was stopped.
cyclophosphamide 15632 her serum sodium. She was discharged home with feeding via the gastrostomy tube.After six pulses of cyclophosphamide , she showed marked clinical improvement. Her diplopia had resolved and eye movements had returned to
cyclophosphamide 16320 this treatment, with no progression of cranial disease on MRI, for 20 months following completion of cyclophosphamide . At this point, she had a relapse of ENT disease with nasal blockage and epistaxis requiring a tapering
cyclophosphamide 16561 prednisolone 30 mg daily.Fig. 6Axial intracranial contrast-enhanced T1-weighted MR image after six pulses of cyclophosphamide . There is reduction in the volume of abnormal cavernous sinus enhancing chronic inflammatory tissue
cyclophosphamide 32900 methotrexate treatment at the time of her initial presentation. The NORAM trial compared methotrexate with cyclophosphamide for the treatment of early, non-organ threatening systemic disease [[23]]. Whilst there was no significant
cyclophosphamide 33339 more extensive disease or pulmonary involvement. The lack of a difference between methotrexate and cyclophosphamide at 6 months might reflect the fact that patients in both treatment groups were still receiving steroids
cyclophosphamide 33596 follow-up has revealed that patients in the methotrexate arm of the trial received more corticosteroids, cyclophosphamide , and other immunosuppressive agents than those in the cyclophosphamide group [[24]], indicating less
cyclophosphamide 33667 received more corticosteroids, cyclophosphamide, and other immunosuppressive agents than those in the cyclophosphamide group [[24]], indicating less effective disease control with methotrexate compared to cyclophosphamide
cyclophosphamide 33770 cyclophosphamide group [[24]], indicating less effective disease control with methotrexate compared to cyclophosphamide induction therapy. In light of these data and our clinical experience, it is the authors’ personal
cyclophosphamide 34780 escalate immunosuppression at her local hospital.The final issue is the choice of rituximab versus cyclophosphamide . We initially opted for rituximab over cyclophosphamide to reduce the risk of infertility. However,
cyclophosphamide 34836 final issue is the choice of rituximab versus cyclophosphamide. We initially opted for rituximab over cyclophosphamide to reduce the risk of infertility. However, in view of rapid clinical deterioration we commenced cyclophosphamide.
cyclophosphamide 34950 cyclophosphamide to reduce the risk of infertility. However, in view of rapid clinical deterioration we commenced cyclophosphamide . The RAVE and RITUXIVAS studies both showed non-inferiority of rituximab compared to cyclophosphamide
cyclophosphamide 35052 cyclophosphamide. The RAVE and RITUXIVAS studies both showed non-inferiority of rituximab compared to cyclophosphamide in AAV [[25], [26]]. However, the results from these trials cannot necessarily be extrapolated to our
cyclophosphamide 35806 prednisolone) was insufficient. The patient improved markedly after we commenced high-dose corticosteroids and cyclophosphamide . For severe cases where rapid control of disease is necessary, this combination of cyclophosphamide
cyclophosphamide 35906 cyclophosphamide. For severe cases where rapid control of disease is necessary, this combination of cyclophosphamide and rituximab may be preferable to either drug alone. Indeed, in the RITUXIVAS trial i.v. cyclophosphamide
cyclophosphamide 36013 cyclophosphamide and rituximab may be preferable to either drug alone. Indeed, in the RITUXIVAS trial i.v. cyclophosphamide 15 mg/kg was given alongside the rituximab doses in weeks 1 and 3 [[26]].ConclusionsPituitary involvement
cyclophosphamide 36797 involvement. In this case a good outcome was achieved with high-dose steroid and dual treatment with cyclophosphamide and rituximab with minimal adverse events. In cases of severe neurological compromise due to GPA where
desmopressin 15496 of the pituitary (Fig. 4 b&c). She was diagnosed with cranial diabetes insipidus and treated with desmopressin , with normalization of her serum sodium. She was discharged home with feeding via the gastrostomy tube.After
testosterone 25971 with contrast-enhancement.Pituitary stalk thickened & infiltrated.Loss of PSHyperprolactinaemia↓ testosterone ↓ LH, normal FSH↓ cortisol & ACTHNormal TSHConstitutional, polyarthritis, peripheral neuropathy,
Select Disease Character Offset Disease Term Instance
diabetes insipidus 160 polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus : a case report and literature reviewJames E. PetersVivek GuptaIbtisam T. SaeedCurtis OffiahAli S. M.
diabetes insipidus 1145 palsy, palsies of cranial nerves IX-XII (Collet-Sicard syndrome), and the rare complication of cranial diabetes insipidus due to pituitary infiltration. The glossopharyngeal, vagus and accessory nerve palsies resulted in severe
diabetes insipidus 15460 pathological enhancement and diffuse thickening of the pituitary (Fig. 4 b&c). She was diagnosed with cranial diabetes insipidus and treated with desmopressin, with normalization of her serum sodium. She was discharged home with
diabetes insipidus 17033 complications of extensive cranial nerve palsies (involvement of nerves II-IV, V3, and VI-XII) and diabetes insipidus due to granulomatous infiltration of the skull base.Our report highlights several learning points. First,
diabetes insipidus 18215 illustrates central nervous system (CNS) manifestations of GPA, including the rare complication of cranial diabetes insipidus due to pituitary infiltration and compression. In contrast to peripheral nerve involvement, CNS disease
diabetes insipidus 21954 insipidus was the most common endocrine abnormality, occurring in 47 patients (81%). In 4 of these, diabetes insipidus occurred following sellar surgery. Anterior pituitary hormone abnormalities occurred less frequently:
diabetes insipidus 22742 hyperintensity on T1 weighted images was reported in 17 cases, and correlated with the presence of diabetes insipidus . In a few cases, hyperprolactinaemia and the presence of a pituitary lesion on imaging caused diagnostic
diabetes insipidus 22919 pituitary lesion on imaging caused diagnostic confusion with a prolactinoma [[21], [22]]. However, diabetes insipidus and the loss of the normal high signal in the posterior pituitary on T1 weighted MRI are unusual in
diabetes insipidus 32260 normalENTPR3 +veAbbreviations: ACTH adrenocorticotropic hormone, cANCA cytoplasmic pattern ANCA staining, DI diabetes insipidus , GH growth hormone, IGF-1 insulin-like growth factor 1, LH luteinising hormone, FSH follicular stimulating
hypogonadism 22075 occurred following sellar surgery. Anterior pituitary hormone abnormalities occurred less frequently: hypogonadism was present in 32 patients, secondary hypothyroidism in 20, hyperprolactinaemia in 19, and growth hormone
hypopituitarism 26783 renalPR3 +ve5557MPituitary enlargement & central necrosis. Heterogenous enhancement.DIHyperprolactinaemiaPan hypopituitarism Peripheral neuropathy, pulmonary, retinal vasculitis, digital, cerebral and renal infarctsPR3 +veSpisek
hypopituitarism 28820 renalcANCA +ve (−ve by the time of sellar manifestations)Hughes 2013 [[55]]231869611N/A30FSellar massPan hypopituitarism OcularN/APereira 2013 [[56]]22898089148*48FAppearances of pituitary microadenoma, but histopathlogy revealednecrotizinggranulomatousinflammationHyperprolactinaemia↓
hypothyroidism 22126 hormone abnormalities occurred less frequently: hypogonadism was present in 32 patients, secondary hypothyroidism in 20, hyperprolactinaemia in 19, and growth hormone deficiency in 5. Reliable estimation of the frequency

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