poly glandular autoimmune syndroms

CARPENTER SYNDROME - A RARE CASE REPORT

G.Ravi Kiran, Dr.V. Chandrashekar, Dr. Bikshapathi Rao

Department of General Medicine, MGM Hospital

Kakatiya Medical College, Warangal, Telangana

Case Report

A 28 Year old female patient presented with C/C of

Irregular Menstrual Cycles from 2 years,

easy fatigability, Dizziness from 2 months,

she is a known diabetic since 8 years (on human mixtard 30/70 16U/14U),

known epileptic (on carbamazepine since 3 years),

O/E patient is pale, afebrile, PR-114/m. Regular , BP- 90/50 mm hg (supine position), BP - 50/34 mm hg

(Standing Position) & there is Hyperpigmentation of Hands, feet, Axillae, Face

CNS & other Systemic Examination was Normal

INVESTIGATIONS

GRBS (at presentation) was 211mg/dl &

Haemogram: Hb: 7.9 gm % , Microcytic Hypochromic Anaemia, TLC: 5400/mm3, Platelets: 2.1lakh/mm

3,

ESR: 15mm/1st Hour,

1st day FBS: 115 mg/dl, PLBS: 192 mg/dl, HbA1c: 5.4% ,

CXR, USG abdomen: Normal Study ,

RFT & LFT: Within Normal Range,

USG neck shows diffuse thyromegaly, with Free T3:2.0 pg/ml, Free T4:0.444 ng/dl, TSH:11.4ùIU/ml,

FNAC of thyroid: Lymphocytic Thyroiditis & Anti TPO Ab (Immuno ligand Assay) : 260 IU/l

Anti GAD Ab (Chemi luminescent Assay): 189 IU/ml

Serum Electrolytes (Before Treatment) Consistently Na+ (mean 128.1meq/l) & K+ (Mean 5.95meq/l) with

Cl- (Mean 93.6 meq/l) &

Discussion

This triple Endocrine Insufficiency is part of PAS & is Historically labelled as Carpenter Syndrome (part of PAS

II). Polyglandular autoimmune syndromes (PAS) are rare endocrinopathies characterized by coexistence of at

least 2 glandular autoimmune diseases. Types I,II,III,IV are distinguished, Thus, In Cases with 2 or more

endocrinopathies possibility of PAS should be Considered, evaluated & treated Accordingly

early morning Serum Cortisol: 3.9 úg/dl

Serum ACTH level: 277pg/ml

High Dose (250 ug) Cosyntopin test:

•Serum Cortisol (Baseline) 3.9 úg/dl ,

• (After 30 min): 6.8 úg/dl

• (after 60 min): 7.6 úg/dl),

Upper GI endoscopy: Normal Study,

Stool for Occult Blood: -ve &

Monteux test: -ve (<5mm induration)

CECT Brain Shows Calcified Granulomas in

frontal Lobe