Rosie: Limited Systemic Sclerosis

I was diagnosed in 1992 with a presence of positive anti-centromere antibodies (ACA). By 1997 I was unable to maintain paid work. To date there is no curative treatment.

Some of my symptoms may not be due to limited scleroderma, however most of these symptoms have appeared since my diagnosis. Raynaud's began some twenty years ago and has been getting progressively worse; fingertip ulcerations occur regularly; and I have esophagael dysmotility with difficulty swallowing and gastric reflux. I had a laparoscopic Nissen Fundoplication in 2000. I also have sclerodactyly and thickening of facial skin with many telangiectasias on fingers, face, lips and tongue. I have alopecia areata. I have cardiac involvement as seen on my echocardiogram in 1998. I had a follow up echocardiogram in 2003 which showed no significant changes.

I suffer with pain and stiffness in muscles and joints due to the thickening of tendons and joint linings. I also have reduced lung function with impaired gas exchange, which was diagnosed by pulmonary function test (PFT) and CAT scan; the results being consistent with pulmonary vascular disease/lung parenchymal disease consisting of shortness of breath with activity, fatigue and generalized weakness.

In addition to the above problems I also have Sjogren's which has caused dry mouth and dental problems, recurring mouth ulcers and dry nostrils. I have vitiligo on my hands, neck and face. I also have occasional diarrhea/constipation due to thickening of the bowel wall, and mild anemia.

In November 1999, I developed mononeuropathy multiplex (bilateral perennial nerve palsies) which has caused a mild right foot drop. In 1985 I presented with evidence of spondylitis from osteoarthritis. In 1991, right elbow tendonitis which is treated with cortisone injection. In 1995, trochanteric bursitis in my left hip which is also treated with cortisone and physiotherapy; and in 1996 a trapezium was removed from my right thumb due to osteoarthritis. And I have papules on my skin, mainly on upper back and forearms, that itch and result in skin lesions from scratching.

I am currently under the care of Dr. Laurie Clemens, Rheumatologist, at St. Vincent's Hospital in Melbourne since September 8, 2002, and Dr. R. Meyer, a general practitioner (GP) in Mildura. I am taking several medicines to control my various symptoms.

I am currently living in a de facto relationship. My partner, although mostly a caring person and a great support with household activities, does not really understand my condition especially when I can be so cold on a seemingly delightful day, nor does he understand why I do not feel energetic and am unable to keep up with him.

~ Update 1-14-04 ~

I have started a scleroderma support group in Mildura, Victoria, Australia. If you or a loved one are affected by scleroderma or related illnesses or symptoms and live in our area, I invite you to contact me for meeting information.

~ Update 6-19-04 ~

I was diagnosed in 1992 with the presence of positive anti-centromere antibodies.

My clinical features, including symptoms that may not necessarily be due to my scleroderma are as follows:
• Calcinosis of phalanges
• Raynaud's (began some twenty years ago) and has been getting progressively worse
• Fingertip ulceration occurring regularly
• Esophageal dysmotility ; Atony of esophagus with gastric reflux and regurgitation;
• Sclerodactyly ; Skin thickening primarily of distal phalanges;
• Flexion contracture right index finger;
• Telangiectasias on fingers, hands, face, lips, tongue and inside nostrils;
• Vitiligo on hands, neck and face;
• Pulmonary arterial hypertension - shortness of breath with activity, fatigue;
• Interstitial fibrosis of the lungs - impaired gas exchange (ventilation/perfusion mismatch)
• Dental problems (loose teeth) due to thickening of periodontal membrane and alteration of tooth suspensory ligament
• Dry mouth and nostrils
• Recurring mouth ulcers
• Diarrhea/constipation
• Alopecia areata
• Pruritus, primarily upper back, arms and hands;
• Mono neuropathy multiplex (bi-lateral perennial nerve palsies), began in November 1999 with mild right foot drop
• Arthralgia - generalized;1985
• Spondylitis identified due to Osteoarthritis, 1985
• Tendonitis, right elbow, 1991
• Left trochanteric bursitis, 1995
• Left olecranon bursitis, 2003

I am currently on many medications. The investigations that I have gone through are:
• Echocardiogram ~ 1998; 2003;
• Nerve conduction studies ~ 1999 results consistent with mononeuropathy multiplex;
• Esophageal manometry ~ 2000, results consistent with atony of the esophagus;
• CAT scan/PFT scans~ 1998; 2000; 2003 results consistent with pulmonary vascular disease/lung parenchymal disease
• Many pathology tests of blood and urine.

The treatments and surgery I have undergone are:

• In 2004 for an ulcer that progressed to osteomyelitis on second PIP joint, right index finger that was treated with IV Prostaglandin in the first instance followed by intensive IV Flucloxicillin in the second instance.

• In 2003 for an infarction and dry gangrene of the right index finger treated with three IV infusions of Iloprost.

• In 2000, gastro-oesophageal reflux disease from lower gastro-oesophageal sphincter tone alleviated with laparoscopic Nissen fundoplication surgery.

• In 1996 I had a trapezium resected from right thumb and in 1991; 1992; 2003;

• In 2004 I had tendonitis in the right olecranon, which was treated with cortisone injection, as well as bursitis in the left trochanter that was treated with cortisone injections and physiotherapy; and bursitis in the left olecranon.

~ Update Recap 10-23-06 ~

Diagnosed 1992 - presence of positive anti-centromere antibodies. My clinical features: including symptoms that may not necessarily be due to my scleroderma:

Raynaud's (began some twenty years ago) affects both hands and feet
Fingertip ulceration
Oesophageal dysmotility
Atony of oesophagus
Gastric reflux
Sclerodactyly
Skin thickening primarily of distal phalanges
Flexion contracture right index finger
Telangiectasis- fingers, hands, face, lips, tongue, neck
Vitiligo on hands, neck and face
Pulmonary Arterial Hypertension - shortness of breath with activity, fatigue
Impaired gas exchange (ventilation/perfusion mismatch)
Periodontal Ligament Resorption (loose teeth) due to thickening of periodontal membrane and alteration of tooth suspensory ligament.
Dry mouth and nostrils; mouth ulcers
Constipation
Alopecia areata
Pruritus -Primarily upper back, arms and hands, scalp and face
Mononeuropathy Multiplex (Bi-lateral Perennial Nerve Palsies). Began in Nov 1999 with mild right foot drop.
Arthralgia & Fatigue
Chronic Lymphocytic Leukaemia (diagnosed October 2006)

INVESTIGATIONS:

1. Cardiac Echogram ~ 1998; 2003; 2005; 2006
2. Nerve Conduction Velocity tests ~ 1999 results consistent with Mononeuropathy Multiplex
3. Oesophageal Manometry ~ 2000 results consistent with atony of the oesophagus
4. CAT scan/PFT scans ~ 1998; 2000; 2003; 2004 results consistent with pulmonary vascular disease/lung parenchymal disease.
5. Pathology-blood, urine
6. Endoscopies-most recent March 2006
7. Mammogram-October 2006 NAD
8. Pap Smear-May 2006 NAD
9. Dermatologist-December 2005 for Alopecia and Folliculitis lower arms (Novasone Ointment)
10. Plain x-ray followed by CT Scan Left hip, September 2006 -results: ? bone cysts

TREATMENT/SURGERY:
2004 - Ulcer that progressed to Osteomyelitis on 2nd PIP joint, right index finger that was treated with IV Prostaglandin in the first instance followed by intensive IV Flucloxicillin in the second instance.
2003 - Infarction and dry gangrene of the right index finger treated with three IV infusions of Iloprost.
2000 - Gastro-oesophageal reflux disease alleviated with Laparoscopic Nissan Fundoplication.
1996 -Excision of Trapezium from Right Thumb
1991; 1992; 2003; 2004 - Tendonitis Right Olecranon (treated with cortisone injection) - Bursitis Left trochanter treated with cortisone injections and physiotherapy; Bursitis Left Olecranon

As the time passes, fourteen years so far, I am finding life seems to be a continuous struggle to cope with my condition. I do not have much of a support system and I am so tired of battling on my own.