Annals of Medicine and Surgery 80 (2022) 104143

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Annals of Medicine and Surgery

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Case Report

Scleroderma and interstitial lung disease - A case report

Fahad Gul a, *, Amna Siddiqui b, Prakhyath Srikaram c, Nabeela Fatima d
a
Rawalpindi Medical University, Rawalpindi, Pakistan
b
Karachi Medical and Dental College, Karachi City, Sindh, 74700, Pakistan
c
Kurnool Medical College, Kurnool, 518002, Andhra Pradesh, India
d
St Pauls College of Pharmacy, Turkayamjal, 501510, Hyderabad, India

A R T I C L E I N F O A B S T R A C T

Keywords: Background: Systemic sclerosis with interstitial lung disease is one of the rarely reported autoimmune disorders.
Scleroderma The ILD associated with systemic sclerosis is the most common cause of mortality in these patients.
Interstitial lung disease Case presentation: A 37-year-old female patient who is a known case of Scleroderma, Cor pulmonale, and hy­
Systemic sclerosis
pothyroidism presented with the exacerbated symptoms of dyspnea and orthopnea. On examination, she had
digital gangrene as a dermatological complication of systemic sclerosis. The patient was given medical man­
agement and was improving.
Discussion: ILD is the dreaded complication of systemic sclerosis. Pulmonary hypertension that developed sec­
ondary to the ILD in this patient led to the cor pulmonale. The patient has the exacerbation of the same.
Conclusion: Early detection and management of the ILD-SS are very important to prevent progression, exacer­
bations, and morbidity associated with it.

1. Introduction This case report has been reported in line with the SCARE 2020
guidelines [9]. To the best of our knowledge, this is the first detailed case
A chronic autoimmune disease characterized by thickening and report describing the clinical presentation and HRCT findings of a pa­
fibrosis of the skin is known as Systemic sclerosis or scleroderma (SSc). It tient with SSc-ILD in the country. This case aims to raise awareness of
is a connective tissue disorder of unknown etiology, with variable this rare entity and emphasize the need for its early detection, strict
clinical manifestations, chronic and usually a progressive course, which management, and the utter need for the development of safe and
is often presented with multi-organ involvement, including lungs [1]. effective treatments, that are capable of improving outcomes and dis­
Lung fibrosis occurs in approximately four-fifths of patients with SSc; ease progression.
approximately one-fourth develop progressive interstitial lung disease
(ILD), with 10-year mortality of around 40%, making it one of the 2. Case presentation
leading causes of morbidity and mortality [2,3].
Since, SSc is a rare disease across the globe, as well as in the Asian A 37-year-old female patient with known complaints of Scleroderma
population and thus, might be overlooked in clinical practice [4,5]. Its associated with ILD, Cor pulmonale, and hypothyroidism presented to
early diagnosis, is often a source of challenge for medical practitioners, the hospital via OPD with complaints of SOB Grade-IV and orthopnea,
due to its early presentation comprising of, non-specific clinical mani­ which was not accompanied by wheeze. She had a history of cough and
festations such as cough, dyspnea, and chest pain [6]. pain in the fingers of both hands, one year back. Over the last 10 days,
Early diagnosis of SSc-ILD is crucial to initiate treatment and prevent the patient developed breathlessness. Gradually patient complained of
disease progression, thus, High-resolution computed tomography loss of wrinkling over the face and limitation of movement at finger
(HRCT) of the chest is recognized as a modality of choice for diagnosing joints due to skin tightening, accompanied by pain in the digits. The
and assessing SSc-ILD [7]. Further, the latter demands familiarity with patient’s drug history, allergy history, family history, and psychosocial
HRCT findings and thorough clinical examination. history were not significant.

* Corresponding author. Rawalpindi Medical University, Holy Family Rd, Block F Block E Satellite Town, Rawalpindi, Pakistan.
E-mail addresses: [email protected] (F. Gul), [email protected] (A. Siddiqui), [email protected] (P. Srikaram), nab.hameed25@
gmail.com (N. Fatima).

https://doi.org/10.1016/j.amsu.2022.104143
Received 15 May 2022; Received in revised form 1 July 2022; Accepted 6 July 2022
Available online 9 July 2022
2049-0801/© 2022 Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY license
(http://creativecommons.org/licenses/by/4.0/).
F. Gul et al. Annals of Medicine and Surgery 80 (2022) 104143

On inspection, findings were, diffuse skin pigmentation, with diaphragm-effusion while right costophrenic angle and right dome of
particularly salt and pepper pigmentation found in the face and neck the diaphragm were normal which depicted that the left lung was more
region, impending digital gangrene of, the index fingers bilaterally and involved than the right one in our patient (Fig. 2).
an ulcer noted on the right foot (Fig. 1). On general and systemic ex­ Skin involvement is one of the earliest and most frequent manifes­
amination, findings were unremarkable, except in RS findings, bilateral tations of scleroderma, so patients encounter dermatologists as first-line
crept were heard. doctors. Consistent with this criterion our patient presented with salt
Moving forward, she had a history of nail removal of the index finger and pepper pigmentation of the face and neck, diffuse skin pigmenta­
one week back and developed wounds in the area that were progressive. tion, and bilateral index finger gangrene representing the advanced
On further evaluation, the patient is on a home oxygenator. The 2D stage cutaneous manifestation.
echocardiography showed pulmonary hypertension with a right atrium The patient also developed pulmonary hypertension secondary to
and ventricular dilation accompanied by severe tricuspid regurgitation. interstitial lung disease. Pulmonary hypertension had progressed to such
High Resolution Computed Tomography (HRCT) of the chest was done an extent to cause right heart failure in this patient. This is called cor
which revealed decreased volume of both lungs with diffuse ground pulmonale. This can be evidenced by the 2D Echo showing the findings
attenuation and traction bronchiectasis and bronchiectasis in bilateral of the right atrium and ventricular dilation with severe tricuspid
lung fields. Notably, subpleural interlobular and intralobular septal regurgitation.
thickening with increased reticulation was found, along with focal areas Management of the ILD is mainly with corticosteroids. Symptomatic
of early honeycombing-that is suggestive of ILD-fibrotic non-specific management is warranted if the patient has associated conditions. The
interstitial pneumonia (NSIP) pattern. Hepatomegaly with an elevated literature recommends the usage of Mycophenolate mofetil in the
dome of the diaphragm was also spotted. management of SS-ILD in addition to corticosteroids [8]. The patient
The diagnosis is made based on his findings of bilateral thickening of was put on medications for pulmonary hypertension like Ambrisenton,
fingers on both hands extending up to metacarpophalangeal joints as per an endothelin antagonist which inhibits vasoconstriction, and sildenafil,
the 2013 American College of Rheumatology (ACR) criteria. Conclu­ a PDE5 inhibitor that promotes vasodilation, especially in pulmonary
sively, the above findings gave us a diagnosis of diffuse scleroderma with arteries. The prompt management of pulmonary hypertension treats the
ILD-exacerbation. cor pulmonale and its exacerbations subsequently. The patient was also
The patient was treated with Inj. Cefpirome 1g IV BD, Tab.Levoce­ put on torsemide and spironolactone which is a combination of loop
trizine + Monteleukast, Tab. Torsemide, Tab. Sildenafil, Tab.Myco­ diuretic and potassium-sparing diuretic to treat fluid backup in cor
phenolate 500mg PO BD, Tab.Hydrocortisone 50mg IV BD, Tab.Aspirin, pulmonale. The patient has a satisfactory course in the hospitalization
Tab. Ambrisentan, Tab. Gabapentin 100ng PO OD, Tab.Prazosin 1mg PO and was discharged with almost the same medications and routine
BD during the hospital stay. She showed significant improvement and follow-up.
was discharged in stable condition.
4. Conclusion
3. Discussion
A 37-year-old female is diagnosed with systemic sclerosis - ILD. The
Our patient presented with acute exacerbation of symptoms of gold standard non-invasive diagnosis can be made with HRCT which can
scleroderma-associated interstitial lung disease complicated by cor record even the early changes. Management is mainly with corticoste­
pulmonale. CT chest has shown ground attenuation, traction bronchi­ roids and supportive medications. Early detection and prompt man­
ectasis, septal thickening, and honeycombing which are consistent with agement are needed to prevent morbidity and mortality.
standard findings of interstitial lung disease in HRCT which is consid­
ered the most sensitive and gold standard modality in the diagnosis of Sources of funding for your research
SSc-ILD and has the utmost importance in monitoring disease prognosis.
Despite established guidelines to use HRCT for SSc-ILD diagnosis only None.
66% of SSc experts are using it. Lack of clinical experience, lack of
adequate knowledge, radiation exposure, imaging cost, and feasibility
may be among the factors in its hindrance [2]. Chest x-ray AP view
showed characteristic patchy consolidation in the left mid and lower
lobe with ill-defined left costophrenic angle and left dome of

Fig-1. Dermatologic findings. Fig-2. X-ray findings.

2
F. Gul et al. Annals of Medicine and Surgery 80 (2022) 104143

Ethical approval Declaration of competing interest

Ethical approval was not required as per country guidelines. No conflict of interest to be declared.

Consent Acknowledgement

Written informed consent was obtained from the patient for publi­ Special mention to the Peer Research Mentorship Program (PRMP)
cation of this case report and accompanying images. A copy of the initiated by the International Society Of Chronic Illness for their support.
written consent is available for review by the Editor-in-Chief of this
journal on request. References

Author contribution [1] R. Giacomelli, V. Liakouli, O. Berardicurti, P. Ruscitti, P. Di Benedetto, F. Carubbi,

G. Guggino, S. Di Bartolomeo, F. Ciccia, G. Triolo, P. Cipriani, Interstitial lung
disease in systemic sclerosis: current and future treatment, Rheumatol. Int. 37 (6)
Fahad Gul: Study concept, data collection, interpretation, manu­ (2017 Jun) 853–863.
script writing, Amna Siddiqui: Study concept, data collection, inter­ [2] D. Khanna, D.P. Tashkin, C.P. Denton, E.A. Renzoni, S.R. Desai, J. Varga, Etiology,
risk factors, and biomarkers in systemic sclerosis with interstitial lung disease, Am.
pretation, manuscript writing, Prakhyath Srikaram: Study concept, J. Respir. Crit. Care Med. 201 (6) (2020 Mar 15) 650–660.
Interpretation, manuscript writing, Dr. Nabeela Fatima: Study concept, [3] A. Perelas, R.M. Silver, A.V. Arrossi, K.B. Highland, Systemic sclerosis-associated
data collection, interpretation, manuscript writing. interstitial lung disease, Lancet Respir. Med. 8 (3) (2020 Mar 1) 304–320.
[4] B. Ranque, L. Mouthon, Geoepidemiology of systemic sclerosis, Autoimmun. Rev. 9
(5) (2010 Mar) A311–A318.
Registration of research studies [5] M. Bairkdar, M. Rossides, H. Westerlind, R. Hesselstrand, E.V. Arkema,
M. Holmqvist, Incidence and prevalence of systemic sclerosis globally: a
comprehensive systematic review and meta-analysis, Rheumatology 60 (7) (2021
1Name of the registry: Not Applicable
Jul) 3121–3133.
2 Unique Identifying number or registration ID: Not Applicable [6] J.H. Chung, C.M. Walker, S. Hobbs, Imaging features of systemic sclerosis-associated
3 Hyperlink to your specific registration (must be publicly accessible interstitial lung disease, JoVE 16 (160) (2020 Jun), e60300.
and will be checked): Not Applicable [7] N.S. Valeur, A.M. Stevens, M.R. Ferguson, E.L. Effmann, R.S. Iyer, Multimodality
thoracic imaging of juvenile systemic sclerosis: emphasis on clinical correlation and
high-resolution CT of pulmonary fibrosis, AJR Am. J. Roentgenol. 204 (2) (2015
Guarantor Feb) 408–422.
[8] A. Fischer, K.K. Brown, R.M. Du Bois, S.K. Frankel, G.P. Cosgrove, E.R. Fernandez-
Perez, T.J. Huie, M. Krishnamoorthy, R.T. Meehan, A.L. Olson, J.J. Solomon,
Dr. Nabeela Fatima. Mycophenolate sdisease, J. Rheumatol. 40 (5) (2013 May 1) 640–646.
[9] R.A. Agha, T. Franchi, C. Sohrabi, G. Mathew, A. Kerwan, A. Thoma, et al., The
Provenance and peer review SCARE 2020 guideline: updating consensus surgical CAse REport (SCARE)
guidelines [Internet], Int. J. Surg. 84 (2020 Dec 1), 226–30, https://pubmed.ncbi.
nlm.nih.gov/33181358/. (Accessed 27 June 2022).
Not commissioned, externally peer-reviewed.