Diagnosis of Primary Hypophysitis in Germany.
BACKGROUND
Representative data on diagnostic findings in primary hypophysitis (PrHy) are scarce.


OBJECTIVE
The objective of the study was to collate consistent data on clinical features in a large series of patients with PrHy. Another objective was to gain information on current practice in a diagnostic work-up.


METHODS
The Pituitary Working Group of the German Society of Endocrinology conducted a nationwide retrospective cross-sectional cohort study in Germany.


METHODS
Seventy-six patients with PrHy were identified.


METHODS
Clinical and endocrinological features were assessed.


RESULTS
Headache (50%) and increase in body mass (18%) were the most frequent nonendocrine symptoms. Hypophysitis was associated with pregnancy in only 11% of the female patients. Diabetes insipidus was found in 54% of the patients at presentation. Hypogonadotropic hypogonadism was the most frequent endocrine failure (62%), whereas GH deficiency was the least frequent (37%). With 86%, thickening of the pituitary stalk was the prevailing neuroradiological sign. Compared with surgical cases, the cases without histological confirmation presented more often with suprasellar lesions and had less severe nonendocrine symptoms. Granulomatous hypophysitis was associated with more severe clinical symptoms than lymphocytic hypophysitis. Examination of cerebrospinal fluid was predominantly performed in participating neurosurgical centers, whereas thyroid antibodies were almost exclusively assessed in endocrinological centers.


CONCLUSIONS
In contrast to the literature, hypogonadism was found to be the most frequent endocrine failure in PrHy. Weight gain was identified as a clinical sign of PrHy. In the majority of patients, PrHy can be reliably identified by characteristic clinical signs and symptoms, obviating histological confirmation. The diagnostic approach should be standardized in PrHy.
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Jürgen
J
Honegger
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Sven
S
Schlaffer
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Christa
C
Menzel
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Michael
M
Droste
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Sandy
S
Werner
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Ulf
U
Elbelt
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Christian
C
Strasburger
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Sylvère
S
Störmann
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Anna
A
Küppers
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Christine
C
Streetz-van der Werf
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Timo
T
Deutschbein
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Mareike
M
Stieg
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Roman
R
Rotermund
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Monika
M
Milian
Department of Neurosurgery (J.H., M.M.), University of Tuebingen, 72076 Tuebingen, Germany; Department of Neurosurgery (S.Sc., C.M.), University of Erlangen-Nuremberg, 91054 Erlangen, Germany; Endocrine Practice (M.D., S.W.), 26122 Oldenburg, Germany; Department of Endocrinology, Diabetes, and Nutrition (U.E., C.S.), Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany; Department of Medicine IV (S.St., A.K.), Campus Innenstadt, University of Munich, 80336 Munich, Germany; Division of Endocrinology and Diabetes (C.S.v.d.W.), RWTH Aachen University, 52074 Aachen, Germany; Endocrine and Diabetes Unit (T.D.), Department of Medicine I, University Hospital Würzburg, 97080 Würzburg, Germany; Max Planck Institute of Psychiatry (M.S.), 80804 Munich, Germany; Department of Pituitary Surgery/Interdisciplinary Endocrinology (R.R.), UKE Hamburg, 20246 Hamburg, Germany; and ENDOC Center for Endocrine Tumors (S.P.), 20357 Hamburg, Germany.
Stephan
S
Petersenn
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000069281:Autoimmune Hypophysitis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D003430:Cross-Sectional Studies; D003919:Diabetes Insipidus / Q000209:etiology* / Q000473:pathology; D005260:Female; D005858:Germany; D006801:Humans; D007006:Hypogonadism / Q000209:etiology* / Q000473:pathology; D007018:Hypopituitarism / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D008875:Middle Aged; D010902:Pituitary Gland / Q000473:pathology*; D012189:Retrospective Studies; D055815:Young Adult