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Suggest Treatment For MCTD And Biliary Cirrhosis Symptoms
Question: Respected Sir,
Good evening.
I am giving hereunder a detail history of my wife's illness. I/my wife earlier took advices from number of doctors of Healthcare Magic about a year back. I am in a dilemma and confused whether I should stop her taking from Tab Azathiprine or reduce the dose or continue the same dose of 50mg BD i.e. 100mg daily.
Except bilateral shoulder joint restriction/pain, she is substantially in better condition till now. I earnestly solicit your valuable opinion/advice in this regard.
I request you to kindly take an additional pain to read the following detail case history and then review. I am not in a hurry, no problem if it takes time for some days.
Patient :-
MRS xxxxxxxxxxxxxxxxx, F/46 Yrs, YYYY, XXXXXXX Wt- 51kg, Ht 158cm
Present complaint: Bi-lateral shoulder joint restriction/pain for last about 1(One) year.
History : Body Pain / Joints Pain / Muscle Pain/Movement difficulty (Diagnosed mild post osteophytes formation in cervical 5th - 6th region, early osteoarthritis changes in L4-L5 vertebra, mild anterior and posterior osteophytic lipping in C5- C6 vertebra, I.V. disc space may be minimally diminished at L4-L5 segments) for years together ( from the year 1999). Rheumatoid Arthritis Factor – Negative(done several times in different Labs during last about 15 years)).
Family History : Mother(Osteoporosis/Osteoarthritis/Spodylosis, RA+), 1(Chrone’s disease), Sister 2(Rheumatoid Arthritis RA+)/Osteoporosis/ Osteoarthritis).
Sudden onset : (A) Last year( July to September 2014) suffered swelling of face and limbs, generalized pain and weakness, throat pain with swallowing difficulty, whole body itching, mouth ulcer, joints movement restriction, jaw movement restriction developed gradually before starting any medicine. Doctors consulted – initially local physician and then one to another Dr B L Bala(Medicine) Dr Avijit Roy(Physician) Dr P K Saha(Dermatology) Dr Sekhar XXXXXXX (Medicine) Dr Sekhar Bandopadhya(ENT) Dr XXXXXXX Sarkar(Homeopathy). After all primary diagnostic procedures(CBC, LFT, UrineRE/ME, Creatinine, USG of whole abdomen, Urea, Uric Acid, ECG, HCV, HBSAg, TSH, Vitamin D screening , CPK, CRP, CK-MB even some more than once in different Labs and then according to the treating doctors, nothing significant except mild anemia(Hb% 10.5), high Eosinophil, Vitamin D deficiency, Triglyceride 265, HDL 60, Absolute Eosonophilic Count 369 and CPK 239 were seen. No diagnosis could be made except Eusonophilia. Primary and symptom based medicines could not do anything and conditions was deteriorating gradually. On the basis of clinical manifestation of the patient and physical examinations, local Doctors had suspected MCTD/SLE/Scleroderma/ Dermatomyositis like some auto-immune disease. Then almost all auto-immune and RA related tests (ANA/Anti-CCP/Scl-70/Anti dsDNA, Anti-U1 RNP, ASO Titre Ratio, RA Factor etc) were done and according to the doctors, all the markers had turned-out negative, though all the clinical symptoms/manifestations were likely to suggest some auto-immune disease(most probably MCTD/SLE/Scleroderma). Patient’s condition was very painful due to swelling of limbs/face, whole body itching, generalized pain/weakness, joints restrictions, throat pain/swallowing difficulty, mouth ulcer, restlessness, jaw movement restriction continued for a long period ( middle of July to end September 2014, about 2 months ). By this time skin and muscle had started to be hard/stiff/tight. Took some medicines as prerscribed – Tab Avil 25 for 2 days, Tab Eto-Shine 2 days, Hetrazen 100 for 16 days, Tab Paracetamol 500mg for 3 days , Tab Atarax 25 for 3 days, Tav Xevor 25 for 5 days, Calamina & Dermadew(Aloevera) Skin Lotion continuously for 15 days, but there was no relief of pain and/or itching/itchy sensation or any other difficulties.
(B) Finding no alternative and helplessly, in this condition patient was rushed to limited financial capacity and after huge expenditure already incurred. She had returned on 21/09/2014 by Air without any treatment from there in a very serious condition with whole body swelling started, skin became more hard/tight/glossy and muscles of lower/upper limbs became stiff, itching/pain/movement restriction increased more. In XXXXXXX Doctors’(Medicine/Dermatology/Endocrinology) clinical observations were ( 0n 11/09/2014 - 19/09/2014) were “Polyarthralgia, Swelling of face & limbs, Jaw movement restriction, Non-specific myalgia, Mouth ulcer, Swallowing difficulty, Rash, Lesion on neck”. After clinical check-up and systemic review in the name of ‘Executive Health Check-up’ ( a basket of pathological/radiological tests related to Blood, Heart, Lungs, Chest, Liver, Kidney etc), viewed that it was most probably a case of Scleroderma(with PM/DM overlap) or MCTD though reports did not give anything adverse so significantly except slight low Hb(10.5%), high Eosinophil(55)/ESR(48), Vitamin-D deficiency, TSH(6.5), Cholesterol 140, HDL 40, LDL 23 and Triglyceride 278. Then all advance method of Auto-immune tests including ANA Profile, Anti dsDNA, XXXXXXX ENA IgG Test(Immunoblot) and a Skin Biopsy(Needle) for histopathology and direct immuno-fluorescence were done along with Lungs/Kidney/Chest/Heart related more invasive tests like CT Scan of Chest, PFT, HRCT, Cardiac Echo, Spot Urine Protein/Creatnine ratio & reports came one after one turned-out any possibility/chance of MCTD/SLE/Scleroderma . According to the treating doctors, report of a Skin Biopsy is only conclusive for diagnosis of such diseases but the same was also not suggesting any of those diseases.
Skin Biopsy report of Apollo XXXXXXX - Mild dermal fibrous expansion is present. Few necrotic keratinocyutes are present in the epidermis.Superficial perivascular round cell infiltrates with mild pigment incontinence and mild increase in interstitial mucin. Immuno-fluorescence negative.
CT scan of Chest Plain report of Apollo XXXXXXX - Fibrotic strands in both lung fields, related to sequelae of prior infection. Several small axillary lymphnodes area seen bilaterally upto 1.5 cm in short axis diameter.
HRCT and PFT at Apollo XXXXXXX - No ILD. No Pulmonary Arterial Hypertension.
Final observation/diagnosis by Apollo XXXXXXX : Anemia, Hypothyroidism, Dyslipidaemia, Vit-D deficiency and Eosonophilia. Medicines prerscribed - Tab Hetrazen 100mg, Tab Complete TD, Cap Globac TZ, Tab Tayo, Thyronorm 50mcg, Tab Livogen Z and Tab Absolute 3G to continue till review after 2 months.
Last day comments of doctors at Apollo XXXXXXX : (a) Medicine Specialist – It is a case of Dermatology. (b) Doctor of Skin(Dermatologist) – Although our primary diagnosis was ‘Scleroderma’ but final review is Patient’s present complaints are due to hypothyroidism. (c) Doctor of Thyroid(Endocrinologist) : TSH is slightly high and it may be mild hypothyroidism, normally no medicine is required, still Tab Thyronorm 50mcg is given. Patient’s present complaints are not at all due to thyroid alone.
Being dishearten, she had returned by Air to Siliguri(20/09/2014). After 3 days of taking those medicines, conditions deteriorated and patient’s mental condition also broke down due to continuous whole body itching and pain for 2 months. Dr(Mrs) Arundhoti Dasgupta(one of the renowned Endocrinologists) and Dr Joydeep Dey(Senior Neurologist) at Neotia Getwel Hospital, XXXXXXX were contacted through some well-wisher lady very close to the patient. Primarily they had also noticed Sclerodermatous symptoms (23/09/2014) and had advised for admission noting very serious condition of the patient. As per doctors’ clinical observations that time, patient had Swelling and induration over extremities and face sparing digits, Tightness of skin, microstomia, bilateral pedal edema – pitting, induration of face/back/extrimities sparing digits. She was admitted there last year for 25 days (26/09/2014 to 22/10/2014 under Dr Joydeep Dey(Neurologist) and Dr Arundhoti Dasgupta(Endocrinologist) and was closely monitored by a group of other XXXXXXX doctors of Medicine, Dermatology, Gynecology, General Surgery and Gastroenterology and they undertook some auto-immune screen like ANA, Anti-centromere and Scl-70 to Ab, which were also negative and auto-immune screening earlier done outside more than once had also turned-out negative, but patient’s clinical symptoms were strongly suggesting Scleroderma. By pathological tests, doctors there found high Eosonophilia, ESR and high Absolute Eosonopil Count(1660) and as per treatment/check-up outside/elsewhere, she had Anemia, Vid-D deficiency, Dyslipidemia, Hypotheroidism and Eosonophilia. As Absolute Eosonophilic Count was very high, the patient had already diagnosed to be Eosonophilic and certain Scleromotous symptoms were present but no Reynaud Phenomenon and during physical examinations /clinical check-up outside/elsewhere by number of XXXXXXX specialist doctors had also hinted/suspected possibility of MCTD/SLE/Scleroderma, they had primarily suspected most probably she had Eosonophilic Faciitis with overlap syndrome of
Scleroderma and had treated her with Pulse Methyle Prednisolone 1g each I.V. for
consecutive 5 days. Dr Joydeep Dey(senior Neurologist) in association with other concerned doctors associated with him gave this treatment empirically due to patient’s serious condition and immediately treatment responded significantly and she was relieved substantially. Before starting steroid, samples for Muscle Biopsy and Skin Biopsy(Punch) were drawn and referred to Lab. She was discharged with Oral Steroid took for last 1(One) year (start 40mg and reduced gradually to zero) and is continuously on Tab Azoran 50mg start OD and after 4 months increased to 50 twice daily. Vitamin D Inj one was given at the time of admission initially. Other medicines were CCM 500mg x 2 (Calcium + Vit D3 + Folic Acid) OD, Tab Eosmoprazole 40mg OD, Multivitamin/Mineral/Anti-oxidants Tab each for 6 months and Tab Primosa 1000 OD for 2 months. She was followed up periodically by Dr Joydeep Dey(Neurologist), Dr(Mrs) Arundhoti Dasgupta(Endocrinologisdt) and Dr Saumitra Saha(Surgeon) and CBC, LFT, Kidney function, TSH tests were done periodically and nothing signicantly adverse was noticed. Patient and her relatives ultimately became happy and relieved by the treatment till now. She also took advice of Dr A K Sasmal(Ortho), Dr D K Timsinha(Skin) and Dr Indranath Ghosh(Medicine) while on Steroid/Azoran. For shoulder joint pain/restriction Dr Sasmal(Ortho) had advised only Physiotherapy and done for 2(Two) months continuously but no significant improvement. Primary doctors ( Dr Joydeep Dey & Dr Arundhuti Dasgupta) after their treatment have once also suggested to get opinion of a Rheumatologist.
Skin Biopsy & Muscle Biopsy report in Neotia Getwel, XXXXXXX before treatment started – No significant faciitis and no eosinophil infiltration. Other features are corroborative with Scleroderma. No unequivocal evidence, yet features show possibility of Scleroderma and Dermatomyositis.
Patient is presently taking medicine - Tab Azoran 50mg BD. Tab Thyronorm 25
Present complaint – Bi-lateral shoulder joint pain and restriction of arms movement for about a year. No improvement by Physiotherapy. Slighly puffy face and mild weakness of muscles. Dr XXXXXXX Banik has been consulted on 28/10/2015. He advised to get now her case reviewed by a Rheumatologist first and after getting that opinion he will follow-up.
After treatment by Pulse Methyle Prednisolone, Oral Steriod and adding Tab Azoran 50mg BD, her status has been periodically reviewed by the doctors and several pathological tests related to Blood, Liver, Kidney etc have been done periodically and nothing adverse has so far been noticed by the doctors.
Immuno-suppressant medications course summarized below :-
1. Pulse Methyle Prednisolone - I.V. 1gm each x 5 days ( 30/09/2014 to 04/10/2014)
2. Oral Steroid (Tab Wysolone 40mg) - 2½ months (10/10/2014 to 06/12/2014) and then tapered off/reduced slowly ( 30mg/25mg/22.5mg/20mg/17.5mg/15mg/12.5
mg/10mg/7.5mg/5mg(each dose for about one month) and stopped/withdrawn in middle of last month(October 2015).
3. Tab Azoran(Azathioprine) (NSAID) – 50mg OD x 4 months(December 2014 to March 2015) and 50mg BD i.e. 100mg daily continued till now.
Recent Consultation with Rheumatologist - (02/11/2015 & 05/11/2015)
Adv : 1) Stop Tab Azoran (Azathioprine)
2) Cont Tab Levothyyroxine 50mcg
3) Cont Tab Calcium + Vitamin D3
4) Check ANA/ANF Profile Done awaiting report after 15 days.
5) Vitamin- 25 D3 Screening - do -
Plan : 1) Restart Tab AZA(Azoran) in reduced dose(50mg)
2) Re-check/re-evaluation ANA profile after 1 month
3) Re-check Blood for WBC count after 10 days(15/12/2015)
Note : 1) I have not stopped Tab Azoran(Azathioprine) but reduced to 50mg OD.
2) Planned to follow all other above advices.
Last Five reports :
02/04/2015
Hb% 12.0, RBC 4.11
WBC 7.40, Platelet
1.59, ESR 09mm,
Neutro 52%, Lymph
38%, Monoc 08%, Eosi-
noph 02%, Basoph 00% Total Bili 0.60
D. Billi 0.11
ID Billi 0.49
Protein 5.60
Alb 3.71
Glb 1.91
SGOT 30
SGPT 49
Lactate Dheh
189
(N 81-234) Urea 27mg
Creatinine 0.75
Na 136, K 4.00
Chloride 99.60
Alk Php 115 Ca 9.40
02/04/2015
Hb% 11.2, RBC 4.16
WBC 6.58, Platelet
1.56, ESR 50mm,
Neutro 76%, Lymph
20%, Monoc 02%, Eosi-
noph 01%, Basoph 01%
Study: WBC-Toxic granules in few as PMNs show. Total Bili 0.77
D. Billi 0.40
ID Billi 0.37
Protein 5.62
Alb 3.71
Glb 1.91
SGOT 21
SGPT 26
Urea 27mg
Creatinine 0.75
Na 133, K 3.60
Alk Php 170 u/L
Urine :
Protein : Present
Epi Cell : 3-6/hpf
Puss Cell : 2-3/hpf
TSH 2.60
Ca 9.70mg
FBS 65mg
30/05/2015
Hb% 10.90 RBC 4.13
WBC 5.67 Platelet 1.90
ESR 20mm
Neutro 74%, Lymph
20%, Monoc 02%, Eosi-
noph 03%, Basoph 01%
Study: Normal Total Bili 0.58
D. Billi 0.26
ID Billi 0.32
Protein 5.72
Alb 3.80
Glb 1.92
SGOT 19
SGPT 15 Creatinine 0.65
Na 138, K 4.00
Alk Ph 175 u/L
Urine :
Protein : Present
Epi Cell : 3-6/hpf
Puss Cell : 2-3/hpf
Urine :
Protein : Present
Epi Cell : 2-4/hpf
Puss Cell : 5-8/hpf TSH 3.78
Ca 8.20
FBS 95mg
29/10/2015
Hb% 10.40 RBC 3.89
WBC 3.10 Platelet 1.80
Neutro 63%, Lymph
32%, Monoc 01%, Eosi-
noph 04%, Basoph 00%
Study- Leucopenia Total Bili 0.50
D. Billi 0.20
ID Billi 0.30
Protein 6.20
Alb 4.00
Glb 2.20
SGOT 25
SGPT 16 Urea 13mg
Uric Acid 3.6
Creatinine 0.40
Na 137, K 4.00
Alk Ph 93 u/L
Urine :
Protein : Present
Epi Cell : 3-6/hpf
Puss Cell : 2-3/hpf
Urine :
Epi Cell : 1-2/hpf
Puss Cell : 2-3/hpf TSH 2.13
Ca 9.30
FBS 104mg
CPK 62 u/L
RF/RA
Factor
7.5 IU/mL
Blood
(N<14)
(Immuno)
CRP < 1.00
(N<5.0)
03/11/2015
Hb% 11.00 RBC 3.90
WBC 3.48 Platelet 1.65
Neutro 67%, Lymph
17%, Monoc 10%, Eosi-
noph 05%, Basoph 01%
XXXX, Husband of Mrs XXXX (Patient)
Thanking you, with reagrds.
09/11/2015
End
***************************
Good evening.
I am giving hereunder a detail history of my wife's illness. I/my wife earlier took advices from number of doctors of Healthcare Magic about a year back. I am in a dilemma and confused whether I should stop her taking from Tab Azathiprine or reduce the dose or continue the same dose of 50mg BD i.e. 100mg daily.
Except bilateral shoulder joint restriction/pain, she is substantially in better condition till now. I earnestly solicit your valuable opinion/advice in this regard.
I request you to kindly take an additional pain to read the following detail case history and then review. I am not in a hurry, no problem if it takes time for some days.
Patient :-
MRS xxxxxxxxxxxxxxxxx, F/46 Yrs, YYYY, XXXXXXX Wt- 51kg, Ht 158cm
Present complaint: Bi-lateral shoulder joint restriction/pain for last about 1(One) year.
History : Body Pain / Joints Pain / Muscle Pain/Movement difficulty (Diagnosed mild post osteophytes formation in cervical 5th - 6th region, early osteoarthritis changes in L4-L5 vertebra, mild anterior and posterior osteophytic lipping in C5- C6 vertebra, I.V. disc space may be minimally diminished at L4-L5 segments) for years together ( from the year 1999). Rheumatoid Arthritis Factor – Negative(done several times in different Labs during last about 15 years)).
Family History : Mother(Osteoporosis/Osteoarthritis/Spodylosis, RA+), 1(Chrone’s disease), Sister 2(Rheumatoid Arthritis RA+)/Osteoporosis/ Osteoarthritis).
Sudden onset : (A) Last year( July to September 2014) suffered swelling of face and limbs, generalized pain and weakness, throat pain with swallowing difficulty, whole body itching, mouth ulcer, joints movement restriction, jaw movement restriction developed gradually before starting any medicine. Doctors consulted – initially local physician and then one to another Dr B L Bala(Medicine) Dr Avijit Roy(Physician) Dr P K Saha(Dermatology) Dr Sekhar XXXXXXX (Medicine) Dr Sekhar Bandopadhya(ENT) Dr XXXXXXX Sarkar(Homeopathy). After all primary diagnostic procedures(CBC, LFT, UrineRE/ME, Creatinine, USG of whole abdomen, Urea, Uric Acid, ECG, HCV, HBSAg, TSH, Vitamin D screening , CPK, CRP, CK-MB even some more than once in different Labs and then according to the treating doctors, nothing significant except mild anemia(Hb% 10.5), high Eosinophil, Vitamin D deficiency, Triglyceride 265, HDL 60, Absolute Eosonophilic Count 369 and CPK 239 were seen. No diagnosis could be made except Eusonophilia. Primary and symptom based medicines could not do anything and conditions was deteriorating gradually. On the basis of clinical manifestation of the patient and physical examinations, local Doctors had suspected MCTD/SLE/Scleroderma/ Dermatomyositis like some auto-immune disease. Then almost all auto-immune and RA related tests (ANA/Anti-CCP/Scl-70/Anti dsDNA, Anti-U1 RNP, ASO Titre Ratio, RA Factor etc) were done and according to the doctors, all the markers had turned-out negative, though all the clinical symptoms/manifestations were likely to suggest some auto-immune disease(most probably MCTD/SLE/Scleroderma). Patient’s condition was very painful due to swelling of limbs/face, whole body itching, generalized pain/weakness, joints restrictions, throat pain/swallowing difficulty, mouth ulcer, restlessness, jaw movement restriction continued for a long period ( middle of July to end September 2014, about 2 months ). By this time skin and muscle had started to be hard/stiff/tight. Took some medicines as prerscribed – Tab Avil 25 for 2 days, Tab Eto-Shine 2 days, Hetrazen 100 for 16 days, Tab Paracetamol 500mg for 3 days , Tab Atarax 25 for 3 days, Tav Xevor 25 for 5 days, Calamina & Dermadew(Aloevera) Skin Lotion continuously for 15 days, but there was no relief of pain and/or itching/itchy sensation or any other difficulties.
(B) Finding no alternative and helplessly, in this condition patient was rushed to limited financial capacity and after huge expenditure already incurred. She had returned on 21/09/2014 by Air without any treatment from there in a very serious condition with whole body swelling started, skin became more hard/tight/glossy and muscles of lower/upper limbs became stiff, itching/pain/movement restriction increased more. In XXXXXXX Doctors’(Medicine/Dermatology/Endocrinology) clinical observations were ( 0n 11/09/2014 - 19/09/2014) were “Polyarthralgia, Swelling of face & limbs, Jaw movement restriction, Non-specific myalgia, Mouth ulcer, Swallowing difficulty, Rash, Lesion on neck”. After clinical check-up and systemic review in the name of ‘Executive Health Check-up’ ( a basket of pathological/radiological tests related to Blood, Heart, Lungs, Chest, Liver, Kidney etc), viewed that it was most probably a case of Scleroderma(with PM/DM overlap) or MCTD though reports did not give anything adverse so significantly except slight low Hb(10.5%), high Eosinophil(55)/ESR(48), Vitamin-D deficiency, TSH(6.5), Cholesterol 140, HDL 40, LDL 23 and Triglyceride 278. Then all advance method of Auto-immune tests including ANA Profile, Anti dsDNA, XXXXXXX ENA IgG Test(Immunoblot) and a Skin Biopsy(Needle) for histopathology and direct immuno-fluorescence were done along with Lungs/Kidney/Chest/Heart related more invasive tests like CT Scan of Chest, PFT, HRCT, Cardiac Echo, Spot Urine Protein/Creatnine ratio & reports came one after one turned-out any possibility/chance of MCTD/SLE/Scleroderma . According to the treating doctors, report of a Skin Biopsy is only conclusive for diagnosis of such diseases but the same was also not suggesting any of those diseases.
Skin Biopsy report of Apollo XXXXXXX - Mild dermal fibrous expansion is present. Few necrotic keratinocyutes are present in the epidermis.Superficial perivascular round cell infiltrates with mild pigment incontinence and mild increase in interstitial mucin. Immuno-fluorescence negative.
CT scan of Chest Plain report of Apollo XXXXXXX - Fibrotic strands in both lung fields, related to sequelae of prior infection. Several small axillary lymphnodes area seen bilaterally upto 1.5 cm in short axis diameter.
HRCT and PFT at Apollo XXXXXXX - No ILD. No Pulmonary Arterial Hypertension.
Final observation/diagnosis by Apollo XXXXXXX : Anemia, Hypothyroidism, Dyslipidaemia, Vit-D deficiency and Eosonophilia. Medicines prerscribed - Tab Hetrazen 100mg, Tab Complete TD, Cap Globac TZ, Tab Tayo, Thyronorm 50mcg, Tab Livogen Z and Tab Absolute 3G to continue till review after 2 months.
Last day comments of doctors at Apollo XXXXXXX : (a) Medicine Specialist – It is a case of Dermatology. (b) Doctor of Skin(Dermatologist) – Although our primary diagnosis was ‘Scleroderma’ but final review is Patient’s present complaints are due to hypothyroidism. (c) Doctor of Thyroid(Endocrinologist) : TSH is slightly high and it may be mild hypothyroidism, normally no medicine is required, still Tab Thyronorm 50mcg is given. Patient’s present complaints are not at all due to thyroid alone.
Being dishearten, she had returned by Air to Siliguri(20/09/2014). After 3 days of taking those medicines, conditions deteriorated and patient’s mental condition also broke down due to continuous whole body itching and pain for 2 months. Dr(Mrs) Arundhoti Dasgupta(one of the renowned Endocrinologists) and Dr Joydeep Dey(Senior Neurologist) at Neotia Getwel Hospital, XXXXXXX were contacted through some well-wisher lady very close to the patient. Primarily they had also noticed Sclerodermatous symptoms (23/09/2014) and had advised for admission noting very serious condition of the patient. As per doctors’ clinical observations that time, patient had Swelling and induration over extremities and face sparing digits, Tightness of skin, microstomia, bilateral pedal edema – pitting, induration of face/back/extrimities sparing digits. She was admitted there last year for 25 days (26/09/2014 to 22/10/2014 under Dr Joydeep Dey(Neurologist) and Dr Arundhoti Dasgupta(Endocrinologist) and was closely monitored by a group of other XXXXXXX doctors of Medicine, Dermatology, Gynecology, General Surgery and Gastroenterology and they undertook some auto-immune screen like ANA, Anti-centromere and Scl-70 to Ab, which were also negative and auto-immune screening earlier done outside more than once had also turned-out negative, but patient’s clinical symptoms were strongly suggesting Scleroderma. By pathological tests, doctors there found high Eosonophilia, ESR and high Absolute Eosonopil Count(1660) and as per treatment/check-up outside/elsewhere, she had Anemia, Vid-D deficiency, Dyslipidemia, Hypotheroidism and Eosonophilia. As Absolute Eosonophilic Count was very high, the patient had already diagnosed to be Eosonophilic and certain Scleromotous symptoms were present but no Reynaud Phenomenon and during physical examinations /clinical check-up outside/elsewhere by number of XXXXXXX specialist doctors had also hinted/suspected possibility of MCTD/SLE/Scleroderma, they had primarily suspected most probably she had Eosonophilic Faciitis with overlap syndrome of
Scleroderma and had treated her with Pulse Methyle Prednisolone 1g each I.V. for
consecutive 5 days. Dr Joydeep Dey(senior Neurologist) in association with other concerned doctors associated with him gave this treatment empirically due to patient’s serious condition and immediately treatment responded significantly and she was relieved substantially. Before starting steroid, samples for Muscle Biopsy and Skin Biopsy(Punch) were drawn and referred to Lab. She was discharged with Oral Steroid took for last 1(One) year (start 40mg and reduced gradually to zero) and is continuously on Tab Azoran 50mg start OD and after 4 months increased to 50 twice daily. Vitamin D Inj one was given at the time of admission initially. Other medicines were CCM 500mg x 2 (Calcium + Vit D3 + Folic Acid) OD, Tab Eosmoprazole 40mg OD, Multivitamin/Mineral/Anti-oxidants Tab each for 6 months and Tab Primosa 1000 OD for 2 months. She was followed up periodically by Dr Joydeep Dey(Neurologist), Dr(Mrs) Arundhoti Dasgupta(Endocrinologisdt) and Dr Saumitra Saha(Surgeon) and CBC, LFT, Kidney function, TSH tests were done periodically and nothing signicantly adverse was noticed. Patient and her relatives ultimately became happy and relieved by the treatment till now. She also took advice of Dr A K Sasmal(Ortho), Dr D K Timsinha(Skin) and Dr Indranath Ghosh(Medicine) while on Steroid/Azoran. For shoulder joint pain/restriction Dr Sasmal(Ortho) had advised only Physiotherapy and done for 2(Two) months continuously but no significant improvement. Primary doctors ( Dr Joydeep Dey & Dr Arundhuti Dasgupta) after their treatment have once also suggested to get opinion of a Rheumatologist.
Skin Biopsy & Muscle Biopsy report in Neotia Getwel, XXXXXXX before treatment started – No significant faciitis and no eosinophil infiltration. Other features are corroborative with Scleroderma. No unequivocal evidence, yet features show possibility of Scleroderma and Dermatomyositis.
Patient is presently taking medicine - Tab Azoran 50mg BD. Tab Thyronorm 25
Present complaint – Bi-lateral shoulder joint pain and restriction of arms movement for about a year. No improvement by Physiotherapy. Slighly puffy face and mild weakness of muscles. Dr XXXXXXX Banik has been consulted on 28/10/2015. He advised to get now her case reviewed by a Rheumatologist first and after getting that opinion he will follow-up.
After treatment by Pulse Methyle Prednisolone, Oral Steriod and adding Tab Azoran 50mg BD, her status has been periodically reviewed by the doctors and several pathological tests related to Blood, Liver, Kidney etc have been done periodically and nothing adverse has so far been noticed by the doctors.
Immuno-suppressant medications course summarized below :-
1. Pulse Methyle Prednisolone - I.V. 1gm each x 5 days ( 30/09/2014 to 04/10/2014)
2. Oral Steroid (Tab Wysolone 40mg) - 2½ months (10/10/2014 to 06/12/2014) and then tapered off/reduced slowly ( 30mg/25mg/22.5mg/20mg/17.5mg/15mg/12.5
mg/10mg/7.5mg/5mg(each dose for about one month) and stopped/withdrawn in middle of last month(October 2015).
3. Tab Azoran(Azathioprine) (NSAID) – 50mg OD x 4 months(December 2014 to March 2015) and 50mg BD i.e. 100mg daily continued till now.
Recent Consultation with Rheumatologist - (02/11/2015 & 05/11/2015)
Adv : 1) Stop Tab Azoran (Azathioprine)
2) Cont Tab Levothyyroxine 50mcg
3) Cont Tab Calcium + Vitamin D3
4) Check ANA/ANF Profile Done awaiting report after 15 days.
5) Vitamin- 25 D3 Screening - do -
Plan : 1) Restart Tab AZA(Azoran) in reduced dose(50mg)
2) Re-check/re-evaluation ANA profile after 1 month
3) Re-check Blood for WBC count after 10 days(15/12/2015)
Note : 1) I have not stopped Tab Azoran(Azathioprine) but reduced to 50mg OD.
2) Planned to follow all other above advices.
Last Five reports :
02/04/2015
Hb% 12.0, RBC 4.11
WBC 7.40, Platelet
1.59, ESR 09mm,
Neutro 52%, Lymph
38%, Monoc 08%, Eosi-
noph 02%, Basoph 00% Total Bili 0.60
D. Billi 0.11
ID Billi 0.49
Protein 5.60
Alb 3.71
Glb 1.91
SGOT 30
SGPT 49
Lactate Dheh
189
(N 81-234) Urea 27mg
Creatinine 0.75
Na 136, K 4.00
Chloride 99.60
Alk Php 115 Ca 9.40
02/04/2015
Hb% 11.2, RBC 4.16
WBC 6.58, Platelet
1.56, ESR 50mm,
Neutro 76%, Lymph
20%, Monoc 02%, Eosi-
noph 01%, Basoph 01%
Study: WBC-Toxic granules in few as PMNs show. Total Bili 0.77
D. Billi 0.40
ID Billi 0.37
Protein 5.62
Alb 3.71
Glb 1.91
SGOT 21
SGPT 26
Urea 27mg
Creatinine 0.75
Na 133, K 3.60
Alk Php 170 u/L
Urine :
Protein : Present
Epi Cell : 3-6/hpf
Puss Cell : 2-3/hpf
TSH 2.60
Ca 9.70mg
FBS 65mg
30/05/2015
Hb% 10.90 RBC 4.13
WBC 5.67 Platelet 1.90
ESR 20mm
Neutro 74%, Lymph
20%, Monoc 02%, Eosi-
noph 03%, Basoph 01%
Study: Normal Total Bili 0.58
D. Billi 0.26
ID Billi 0.32
Protein 5.72
Alb 3.80
Glb 1.92
SGOT 19
SGPT 15 Creatinine 0.65
Na 138, K 4.00
Alk Ph 175 u/L
Urine :
Protein : Present
Epi Cell : 3-6/hpf
Puss Cell : 2-3/hpf
Urine :
Protein : Present
Epi Cell : 2-4/hpf
Puss Cell : 5-8/hpf TSH 3.78
Ca 8.20
FBS 95mg
29/10/2015
Hb% 10.40 RBC 3.89
WBC 3.10 Platelet 1.80
Neutro 63%, Lymph
32%, Monoc 01%, Eosi-
noph 04%, Basoph 00%
Study- Leucopenia Total Bili 0.50
D. Billi 0.20
ID Billi 0.30
Protein 6.20
Alb 4.00
Glb 2.20
SGOT 25
SGPT 16 Urea 13mg
Uric Acid 3.6
Creatinine 0.40
Na 137, K 4.00
Alk Ph 93 u/L
Urine :
Protein : Present
Epi Cell : 3-6/hpf
Puss Cell : 2-3/hpf
Urine :
Epi Cell : 1-2/hpf
Puss Cell : 2-3/hpf TSH 2.13
Ca 9.30
FBS 104mg
CPK 62 u/L
RF/RA
Factor
7.5 IU/mL
Blood
(N<14)
(Immuno)
CRP < 1.00
(N<5.0)
03/11/2015
Hb% 11.00 RBC 3.90
WBC 3.48 Platelet 1.65
Neutro 67%, Lymph
17%, Monoc 10%, Eosi-
noph 05%, Basoph 01%
XXXX, Husband of Mrs XXXX (Patient)
Thanking you, with reagrds.
09/11/2015
End
***************************
Brief Answer:
Azathioprine to be taken 100 mg/day
Detailed Answer:
Hello, Sir.
I can certainly understand your concerns.
I apologize for the delayed response owing to my hectic work schedule.
I have worked through your query in detail.
My diagnosis favours MCTD along with the possibility of primary bliary cirrhosis.
This PBC needs a rule out considering the persistent symptoms of itching and fatigue will skin changes.
For this, I suggest you to get an AMA -antimitichondrial antibody testing.
Additionally it is advisable to get a colonoscopy for better assessment.
So far your wife had an intensive evaluation and best possible therapy. My thanks to all the involved physicians.
She certainly needs to continue Azathioprine 50 mg bid instead of od.
Update me with her repeat ANA profile(this should include anti Scl 70 and anti CCP) and AMA profile at the earliest.
Post your further queries if any.
Thank you
Azathioprine to be taken 100 mg/day
Detailed Answer:
Hello, Sir.
I can certainly understand your concerns.
I apologize for the delayed response owing to my hectic work schedule.
I have worked through your query in detail.
My diagnosis favours MCTD along with the possibility of primary bliary cirrhosis.
This PBC needs a rule out considering the persistent symptoms of itching and fatigue will skin changes.
For this, I suggest you to get an AMA -antimitichondrial antibody testing.
Additionally it is advisable to get a colonoscopy for better assessment.
So far your wife had an intensive evaluation and best possible therapy. My thanks to all the involved physicians.
She certainly needs to continue Azathioprine 50 mg bid instead of od.
Update me with her repeat ANA profile(this should include anti Scl 70 and anti CCP) and AMA profile at the earliest.
Post your further queries if any.
Thank you
Above answer was peer-reviewed by :
Dr. Chakravarthy Mazumdar
Respected Doctor,
10/10/2015
Good evening Sir. Sorry to intervene you once again. Request that you kindly recheck the case history. Her CBC Test has shown low WBC(3.10) on 29/10/2015. She was on Tab Azathioprine 50mg x OD for 4 months and then presently on 50mg x BD i.e. 100mg daily for last 8 months. Would it be wise to stop Tab Azathioprine or reduce the dose fearing body's susceptibility of infection due to low WBC count/fall of resistance power of body(immuno-suppression) or maintain the same dose or reduce it fearing the disease flare-up/relapse.
If it is advisable to continue Tab Azathioprine, is there now no way to increase WBC or support body's resistance power ?
Thanking you with regards and wish you all a VERY HAPPY DEWALI.
XXXX.
Respected Doctor,
10/11/2015 (Not 10/10/2015)
Good evening Sir. Sorry to intervene you once again. Request that you kindly recheck the case history. Her CBC Test has shown low WBC(3.10) on 29/10/2015. She was on Tab Azathioprine 50mg x OD for 4 months and then presently on 50mg x BD i.e. 100mg daily for last 8 months. Would it be wise to stop Tab Azathioprine or reduce the dose fearing body's susceptibility of infection due to low WBC count/fall of resistance power of body(immuno-suppression) or maintain the same dose or reduce it fearing the disease flare-up/relapse.
If it is advisable to continue Tab Azathioprine, is there now no way to increase WBC or support body's resistance power ?
Thanking you with regards and wish you all a VERY HAPPY DEWALI.
XXXX.
What about the answer of my follow up question ?
What about answer of my follow-up question/further query ?
10/10/2015
Good evening Sir. Sorry to intervene you once again. Request that you kindly recheck the case history. Her CBC Test has shown low WBC(3.10) on 29/10/2015. She was on Tab Azathioprine 50mg x OD for 4 months and then presently on 50mg x BD i.e. 100mg daily for last 8 months. Would it be wise to stop Tab Azathioprine or reduce the dose fearing body's susceptibility of infection due to low WBC count/fall of resistance power of body(immuno-suppression) or maintain the same dose or reduce it fearing the disease flare-up/relapse.
If it is advisable to continue Tab Azathioprine, is there now no way to increase WBC or support body's resistance power ?
Thanking you with regards and wish you all a VERY HAPPY DEWALI.
XXXX.
Respected Doctor,
10/11/2015 (Not 10/10/2015)
Good evening Sir. Sorry to intervene you once again. Request that you kindly recheck the case history. Her CBC Test has shown low WBC(3.10) on 29/10/2015. She was on Tab Azathioprine 50mg x OD for 4 months and then presently on 50mg x BD i.e. 100mg daily for last 8 months. Would it be wise to stop Tab Azathioprine or reduce the dose fearing body's susceptibility of infection due to low WBC count/fall of resistance power of body(immuno-suppression) or maintain the same dose or reduce it fearing the disease flare-up/relapse.
If it is advisable to continue Tab Azathioprine, is there now no way to increase WBC or support body's resistance power ?
Thanking you with regards and wish you all a VERY HAPPY DEWALI.
XXXX.
What about the answer of my follow up question ?
What about answer of my follow-up question/further query ?
Brief Answer:
Taper the dose of Azathioprine
Detailed Answer:
Hello, Sir.
I can most certainly understand your concerns.
I apologize for the delayed response owing to my hectic work schedule.
I have worked through your query in detail.
The WBC count is marginally low in her case.
Yes, it can run a low risk of infection.
But the dose reduction can also cause disease flare ups.
Hence at this point my suggestion would be to reduce evening dose to 25 mg for 4 weeks maintaining morning dose of 50 mg.
After 4 weeks based on disease activity if it improves or remains stable 25 mg evening dose can be eliminated, making only 50 mg OD as maintenance dose.
Such dose should improve the WBC count as well as avoiding disease flares.
Please check with your physician if he shares and validate my view.
Post your further queries if any.
Thank you.
Post your further queries if any.
Thanking you.
Taper the dose of Azathioprine
Detailed Answer:
Hello, Sir.
I can most certainly understand your concerns.
I apologize for the delayed response owing to my hectic work schedule.
I have worked through your query in detail.
The WBC count is marginally low in her case.
Yes, it can run a low risk of infection.
But the dose reduction can also cause disease flare ups.
Hence at this point my suggestion would be to reduce evening dose to 25 mg for 4 weeks maintaining morning dose of 50 mg.
After 4 weeks based on disease activity if it improves or remains stable 25 mg evening dose can be eliminated, making only 50 mg OD as maintenance dose.
Such dose should improve the WBC count as well as avoiding disease flares.
Please check with your physician if he shares and validate my view.
Post your further queries if any.
Thank you.
Post your further queries if any.
Thanking you.
Above answer was peer-reviewed by :
Dr. Chakravarthy Mazumdar
I will think over the possibility of PBC as another option suspected by you later after monitoring her Blood Count and maintaining Tab Azoran as suggested by you and subsequent symptoms. I will also consult with my primary doctors tomorrow with your opinion. I will get back you in due course again.
Thanking you, I am greatly satisfied.
Please keep up all of yours relentless services. It is very helpful.
XXXX
Regards, 12/11/2015, 9.52 P.M.
Thanking you, I am greatly satisfied.
Please keep up all of yours relentless services. It is very helpful.
XXXX
Regards, 12/11/2015, 9.52 P.M.
Brief Answer:
You are welcome
Detailed Answer:
Hello, Sir.
Thanks for your good wishes.
Update me with her health status.
Take care.
You are welcome
Detailed Answer:
Hello, Sir.
Thanks for your good wishes.
Update me with her health status.
Take care.
Note: For further queries, consult a joint and bone specialist, an Orthopaedic surgeon. Book a Call now.
Above answer was peer-reviewed by :
Dr. Chakravarthy Mazumdar
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